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Cheng-Ching > TrueLearn > Flashcards

TrueLearn Flashcards

1
Q

ABCD2 BP scores point, duration score pts, age?

A

60 min - 2 pt, 10-59 min - 1 pt
BP: >=140/90
2 pts if focal weakness
DM, age >60
DAPT if score 4, ASA only if score <4

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2
Q

MS drugs MOA

A

fingolimod-binds sphingosine 1 Phosphate receptors, blocks lymphs leaving lymph nodes
- Headache
- Elevated LFTs
- Cardiac conduction abnormalities
- **Macular vol
- Abdominal distress
- Leukopenia
- Increased risk of infection

natalizumab - Ab vs alpha-4 integrin - “very late antigen 4” - blocks integrin adhesion interactions, inhibits T lymph migration into CNS
- Progressive multifocal leukoencephalopathy (PML-oligodendrocytes)
- Hepatotoxicity
- Fatigue
- Allergic reaction

glatiramer acetate - stimulates myelin basic protein (T suppressor cells)
- Injection site pain Hypersensitivity reaction, Nausea, Edema
-OK in pregnancy

dimethyl fumarate - Nrf2 pathway activation, anti inflammatory; downregulate cytokine
- Flushing
- Nausea and diarrhea
- Angioedema
- Lymphopenia
- Rare cases of PML

ocrelizmab anti CD 20
- URI
- Infusion reaction
- peripheral Edema
- Neutropenia

alemtuzumab - anti CD52 lymphocyte depletion
- Thyroid disease (Alementarium over or under eating)
- Headache
- Rash
- Abdominal distress
- Infusion reaction
- Fever

cladribine,
teriflunomide (pyrimidine synthesis inhibitor-T for thymine) -
- Teratogenic
- Hypersensitivity reactions
- Nausea
- Headache
- Hepatotoxicity

Pregnancy: can use steroids + IVIG for exacerbation
-interferons stop 1 month before conception
-glatiramer acetate ok during all of pregnancy
-interferons ok early pregnancy

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3
Q

Mollaret triangle

A

inferior olivary nuc and red nuc via central tegmental tract
contralateral dentate

dentate to contralateral red nucleus via superior cerebellar peduncle;
red nucleus to inferior olivary nucleus via central tegmental tract
inferior olive to contralateral dentate via inferior cerebellar peduncle

-palatal tremor/myoclonus if ANY lesion
-can Tx benzos
-pontine infarct

Pica-Wallenberg - damage central tegmental tract (no motor weakness; ipsilateral ataxia, ipsilateral face sensation loss; no taste sense; decrease pain temp contralateral)- lateral medulla + cerebellar hemisphere (AICA at level of pons-lateral)
-SCA - cerebellar peduncle, cerebellum (no brainstem Sx)

corticate above red nucleus: disinhibit red nuc, activate rubrospinal tract -> flexor in uppers

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4
Q

Hypersensitivity rxn allele carbamazepine

A

HLA-B1502

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5
Q

sacral sensation

A

cauda equina - saddle anesthesia, can have normal strength and Le tone

Onuf - sphincter motor nuc S2-S4- urinary
-MSA - cause of urinary incontinence - can be presenting sign

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6
Q

CIDP EMG

A

conduction block: prox CMAP amplitude decreased by greater than 50% of distal CMAP
(amplitude distal always > proximal unless supramax stim)

**temporal dispersion: >30% increase in CMAP duration **
from distal to prox stim sites b/c demyelination

-CMAP velocity <30% lower limit normal-block

prolonged distal motor latencies
PROLONGED F wave >20% normal
/ or no F wave+ CMAP amp>20% normal
-(F wave slowing out of proportion to amplitude loss)
-F waves long first change
vs amplitude decrement -> axonal

(AIDP first change ->lose F waves; H reflexes absent (stimulate tibial nerve-achilles reflex - demyelination at level of nerve ROOTs)
-check if IgA deficient before IVIG

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7
Q

Martin Gruber anastomosis

A

ulnar CMAP amplitude decreased by 50% from wrist to elbow
-stimulating median N at elbow + record from ADM abductor digiti minimi - median fibers cross over to innervate ulnar muscles

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8
Q

genitofemoral N, ilioinguinal N

A

genitofemoral N (L1-L2) - 2 branches:
-femoral (anterior medial thigh sense)

-genital /external spermatic N - through inguinal canal - cremaster, anterior scrotum sense
-genital N-inguinal canal with ilioinguinal N
ilioinguinal N + external spermatic N

ilioinguinal: from t12-L1 nerve roots - abdominal pain + medial thigh numbness - neuralgia during pregnancy - goes through superficial inguinal ring

(femoral N through fem triangle inferiorly)

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9
Q

Tics

A

Dx- D2 R antagonists

DA agonists paradoxically help
stimulants don’ts increase tics

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10
Q

kleine-Levin syndrome

A

aka recurrent hypersomnia; ideopathic
-cycles of hypersomnia + hyperphagia + hypersexuality, confusion, apathy, derealization as adolescent - 16 yrs

vs ideopathic hypersomnolence - not cyclical sleep for 11 hours, chronic daytime sleepiness, short sleep latency on MSLT

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11
Q

narcolepsy type 1 vs 2

A

Need 3 months excessive daytime sleepiness

type 1- narcolepsy + cataplexy
-low hypocretin-1 in CSF OR cataplexy
-sleep hallucinations
-HLA-DQB1*0602 gene

-loss orexin= hypocretin from lateral hypothalamus

type 2 - no cataplexy, normal hypocretin-1 in CSF

Dx: polysomnigram + multiple sleep latency test

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12
Q

rabies

A

-encephalitis, hydrophobia, seizures, N, V, agitation
-temporal lobes, limbic system

Negri body: dark oval large spot around perimetry of cell; can be ultiple

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13
Q

Herpes encephalitis

A

Cowdrey A
-intranuclear, solitary, surrounding halo - large pink blob

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14
Q

Hirano body

A

Alhzheimer’s
-aggregate actin proteins

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15
Q

EPS

A

from D2 R block

acute dystonia - IV diphenhydramine - 5 days drug exposure

Torticollis - Tx Botox (torticollis rotate to side vs laterocollis - head tilt)

Akathisia - inner FEELING-most common D2 EPS
switch to low potency - quetiapine

tardive dyskinesia - 3 months neuroleptic use - chorea = brief, unpredictable abrupt irregular movements
slow writhing - athetosis
slow twisting movements - tardive dystoni

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16
Q

NREM parasomnia

A

Night terrors - arousal, screaming, crying + tachy, diaphoretic; confused when woken up

vs nightmares - patients not confused

vs Panayiotopoulos syndrome - btw 3-6 yrs; +N, +V

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17
Q

ideamotor apraxia

A

localizes left hemisphere

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18
Q

back pain/radiculopathy w/u

A

Imaging not until 6 weeks of Sx

diagnosis: spinal imaging after 6 wks

EMG - confirms Dx
if C6 radic b/c can present like carpal tunnel so need if C6

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19
Q

Mitochondrial disease

A

Kearns Sayre
ragged red fibers on biopsy
-retinitis pigmentosa
-progressive external ophthalmoplegia
-DELETIONS of mitochondrial DNA or rearrangement

Leber hereditary optic neuropathy - point mutations mitochondrial dna

myoclonic epilepsy with RRF - common

Melas - very rare - transfer RNA point mutation

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20
Q

filum terminale causa equina conus medullaris

A

Conus medullaris tapered end of SC then cauda equina nerves

Filum terminale-sack of pia mater, threadlike structure at end of conus medullaris that anchors to cococyx

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21
Q

Mydriasis: Adie’s pupil + CNIII

A

Adie: no light reaction, intact near reaction - light-near dissociation
Mydriasis, tonic pupil, b/c parasympathetic denervation; (chronic)
sluggishly react to light, can accommodate with near response
-
-test: PILOCARPINE -cholinergic - Adie pupil constricts a lot b/c of denevation hypersensitivity
-normal pupil should not constrict with dilute pilocarpine
-little old Adie: pupil small with time

Idiopathic or ciliary ganglion lesions
-react poorly to light but
-after viral infection

CN III palsy - accommodation affected equally; affects E-W fibers in parasympathetics
-CN III palsy: concentrated pilocarpine: constricts CN III palsy

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22
Q

pallister hall syndrome

A

hypothalamic harmartomas
GLI3 gene

-gelastic seizuers - preserved awareness - last 30 sec -refractory to ASMs

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23
Q

Billing medicare/Medicaid

A

No level 1
2 - <30 min new; <20 min old
3-<45 min new; <30 min old
4-<1 hour new; <40 min old

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24
Q

Leukodystrophy

A

CADASIL - NOTCH3 AD - subcortical white matter - strokes, dementia

Alexander - GFAP Chr 17 AD-rosenthal fiber deposits in astrocytic processes - involves U fibers, macrocephaly - BG + thalamus + frontoparietal atrophy

CAnAvAn – aspartoacylase ASPA AR - spongy degeneration includes spinal core involves U fibers, macrocephalic subcortical

Krabbe - AR galactocerebrosidase GALC Chr 14- globous cells (child/adult/adolescent form)-tripnormal early; later atrophy thalamus, splenium, brain stem, IC; vs metachromatic leukodystrophy

Metachromatic leukodystrophy - arylsulfatase A AR- ARSA - spares U fibers, thalamus, splenium, IC; Tigroid pattern, periventricular

Vanishing white matter: eIF-2B-related disorder - translation protein mutation - stressor like illness then hypotonia, ataxia, motor dysfunction - MRI: white matter radiating strips

X L adreno leukodystrophy - Posterior changes on MRI

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25
Q

Tx spasticity

A

-oral meds then intrathecal baclofen - generalized spasticity
GABA B R agonist - baclofen
(Etoh Gaba A)
-risk pump malfunction -> withdrawal/overdose
-withdrawal: seizures, hyperthermia, DIC, rhabdo

-Botox injections
-PT if only mild spasms

refractory - rhizotomy - ablation dorsal rami nerves

(tizanidine - alpha 2 agonist - presynaptic motor nerves; lower BP)

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26
Q

Guyon canal vs cubital tunnel

A

Cubital tunnel - elbow - wrist flexion + addiction weakness
Guyon canal - at wrist -

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27
Q

Palliative care

A

Dying discussion limited by physician not patient or family
-optimal to start palliative care at the time of life threatening or life limited illness

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28
Q

Fregoli vs cotard delusion vs pseudocyesis

A

Fregoli - believe everyone is actually 1 person in various disguises
Cotard - believe they are dead/dying
Pseudocyesis - believe you are pregnant

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29
Q

Benzo wean

A

Reduce by 25% every 2 weeks

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30
Q

Posterior cortical atrophy

A

Alzheimer variant with visual impairment, simultanagnosia, oculomotor apraxia

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31
Q

Pick body vs Lewy body

A

Pick: off center black spot
Lewy body: round spot +/- halo; in old person
-lewy body dementia - quetiapine ok (pimozide typical antipsychotic, don’t use)

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32
Q

Meiosis: Horner + Argyll-Robertson

A

Argyll-R: small pupils don’t react to light,+ react to accommodation

Horner: Dx - interruption sympathetic pathways from hypothalamus
-cocaine drops; inhibit NE reuptake, more NE= Mydriasis (in Horner’s, no Mydriasis; anisicoria more prominent b/c normal pupil larger)
-apraclonidine: Horner pupil larger b/c NE R activated, hypersensitivity in Horner’s causes pupil dilation (normal pupil doesn’t change)

Hydroxyamphetamine: preganglionic lesion dilates, post ganglionic lesion stays same

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33
Q

Aminoacidopathy

A

Nonketotic hyperglycinemia- GLDC, AMT; AR
Early myoclonic encephalopathy
Elevated glycine, NO ketones (vs organic acidemias)

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34
Q

Imaging pregnant vs postpartum

A

No iodine contrast or gad during pregnancy; can use gad if you have to; gad and iodine is ok for breastfeeding

Pregnant: no contrast
Papilledema- get MRV without (MRV+ if post partum even if breastfeeding)

Preeclampsia: get MRI without
PRES: MRI without if pregnant; with contrast post partum

with contemporary CT imaging, attributable risk of cancer to the radiation exposure low
gad ok in breastfeeding

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35
Q

Tetrabenazine

A

VMAT inhibitor
Chorea in Huntington

Vs trihexyphenidyl- anticholinergic - Tx EPS side effects

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36
Q

Meige

A

Botox ONLY; not carbamazepine

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37
Q

Gelastic seizures

A

Refractory seizures lasting 30 sec
Pallister-Hall -GLI3 - hypothalamic hamartoma

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38
Q

Benign myoclonus early infancy

A

Before 1 yr, spasms self limited; awake + alert

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39
Q

Galantamine

A

AChe antagonist+ allosteric nicotinic modulator
Side effects GI

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40
Q

Respiratory patterns

A

Cheyne stokes - BL hemisphere, thalamus
-oscillate btw hyperventilation + hypoventilation
crescendo/decrescendo then apnea, see in sleep
Deep sighing breaths - Kussmaul - DKA
Irregular - ataxia - medulla
Irregular+ shallow - agonal - diffuse anoxic injury

Midbrain (ventral): Central neurogenic hyperventilation - Regular breaths >40 RR , continues during sleep-CNA lymphoma-medial pons

Pons tegmentum - apneustic - prolonged inspiration with 2-3 pause then expiration - SAME APNEAS

Lower Pons (dorsolateral) - cluster breathing - breaths with similar depths then VARIABLE APNEAS
-can see with MSA

Medulla - ataxic - dorsomedial - IRREGULAR in rate, rhythm, amplitude

Lateral medulla, high cervical cord - central neurogenic hypoventilation - loss of respiration during sleep

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41
Q

Vert dissection trauma vs spontaneous vs vert A stenosis

A

Trauma: V3- C1-C2
Spontaneous dissection: V4 - Wallenberg syndrome

Vert A stenosis - V1

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42
Q

storage diseases

A

Fabry - can present in adulthood - renal - amyloid deposition - A Gal who tries ceramics
alpha galactosidase , accumulate trihexyceramide
-pottery wheel splatter: heart + renal (lipid deposits) + angiokeratomas + small fiber neuropathy (hands + foot pedal) +strokes/CV disease (clay clogs)
histo; lipid accumulation in blood vessels

vs gaucher, krabbe - less likely to present as adult

Niemann Pick - foam cells
-A - severe, B; later onset - SMPD1 - sphingomyelin phophosdiesterase-1

type C - NPC1/2 mutations - cholesterol transport

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43
Q

neurocognitive testing - learning disability

A

isolated low memory score - Visual memory

-Stroop - disinhibition, executive fxn

finger tapping, symbol digit coding- psychomotor speed
(abnormal in concussion)

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44
Q

GPi vs DRG vs putamen damage toxicity and treatment

A

GPi - CO, manganese
putamen - methanol + optic nerve
DRG-B6 excess

methanol - Tx ethanol
ethylene glycol - Tx fomepizole in kids, ethanol in adults

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45
Q

trinucleotide repeat expansion

A

CAG - spinocerebellar ataxia (SCA1-ATXN1 and on other genes); Huntington (Caudate has low ACh + GABA) - need 40 repeats for full penetrance

-spinocerebellar ataxia vs Friedreich - can be older, increase DTRs, no sensory loss

GAA-Friedrich’s ataxia - GAAit ataxic (present 5-15, low DTRs but + babinski, sensory loss)

CGG-fragile X - chin (protrudes), giant gonands

CTG-myotonic dystrophy - cataracts, toupee, gonadal atrophy

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46
Q

vascular malformations

A

dural av fistulas - don’t see on MRI -see on angio

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47
Q

patient-physician relations; transition ped to adult care

A

primary non adherence - medication never filled - non fulfillment adherence

secondary non adherence
-non-persistence - start med then stop
-non-conforming - taking med not as prescribed

Patient physician discordance - non adherence - misunderstand doc

Transition to adult care: Transition Readiness Assessment Questionnaire- can bill for; start at age 14; confidential to parents

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48
Q

cerebral contusion

A

-chronic hypodensity - looks like black cystic space
-contre-coup injury

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49
Q

chiari ii vs dandy walker

A

Chiari 0: syringomyelia with mild hernation <3 mm - NORMAL VARIANT

  • chiari I - progressive suboccipital HA-syrinx: can resolve spontaneous with surgical fix in
    -tonisal herniation >5 mm inferior to foramen magnum
    -size of posterior cranial vault prediction of Sx severity, NOT degree of tonsillar herniation
    -McRae line - line at base on foramen magnum - Chari crosses

chiari II: hydrocephalus + myelomeningocele

dandy walker: agenesis cerebellar vermis
iii, iv - no myelomeningocele, only II
chiari iii: cerebellar herniation into encephalocele +/- brainstem

chiari IV: cerebellar hypoplasia/aplasia with normal posterior fossa

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50
Q

split cord 1 vs 2

A

split cord 1: 2 hemicords with own dural sheath, separated by septum

split cord 2: 2 hemicords in 1 dural sheath

sx: tethered cord, gait disturbance, atrophy LE, spasticity

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51
Q

currarino triad

A

anterior sacral meningocele + presacral mass + anorectal abnormality

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52
Q

MEG

A

focal epilepsy on EEG + no structural lesion on MRI

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53
Q

lipomyelomeningocele

A

cutaneous stigmata: hypertrichosis, hyperpigmentation, palpable lipoma

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54
Q

Bunina bodies vs hirano bodies

A

ALS - cytoplasm motor neurons

Hirano: rod shaped, pyramidal CA1 hippocampus
-normal aging or EtOH, Alzheimer’s, Pick’s

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55
Q

breath holding spell; shuddering attack

A

supplement with iron reduces attacks

reproduce: 10 second ocular pressure during EKG->bradycardia/asystole

-incontinence, clonus then sleepy
-no CTH emergent unless prolonged LOC ; can wait for MRI

shuddering attacks: rapid bursts of trembling, benign, normal EEG with myogenic artifact 20-100 Hz
-occur when excited

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56
Q

Parkinson’s management

A

Half life L dopa: 1.5 hours

L-dopa incude dyskinesias Tx:
-amantadine - risk hallucinations - NMDA antagonist
(Sx-constantly needing to move)
-mottled rash - STING RAY

-increase frequency decrease dose L dopa/continuous infusion; continuous apomorphine subcu infusion

-DBS- <75 yrs, **clear response to L Dopa **but dyskinesias or off state; cognitive intact, psych intact
-only partially affective for tremor
-no dementia
-GPi, STN (NOT VIM - for essential tremor)

urinary urgency - Tx mirabegron - beta 3 agonist (less cholinergic vs oxybutynin)

Hallucinations - (infectious w/u) first withdrawal anticholinergic then amantadine then DA agonist

PD psychosis -
Tx - pimavanserin. 5-HT2A R agonist

REM sleep behavior predates LBD + PD
+/- MSA

MRI: ABSENT swallow tail sign = loss of pigmentation in the substantia nigra

PD dementia: RIVASTIGMINE approved

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57
Q

PD drugs

A

pramipexole - DA agonist

Rasagiline - MAO-B inhibitor

Entacapone - COMT

amantadine - NMDA antagonist
-also hastens recovery in comatose pts 16-65 when given 4-16 wks pos-tinjury

L-Dopa side effects - GI upset, nightmares, hallucinations, dizziness

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58
Q

PTSD length Sx, kids; cyclothymic, panic disorder

A

1 month

Sx different in kids vs adolescents - can have disorganized/agitated behavior, not fear
-physical proximity to trauma doesn’t matter

cyclothymic: TWO YEARS Sx

panic disorder: 1 month and one attack, Tx= desensitization and exposure therapy

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59
Q

NCSE vs postictal

A

NCSE - positive symptoms: mydriasis, hiccups, nystagmus, eye deviation

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60
Q

sleep apnea

A

apneas: no respiratory for 10 sec; hypopnea = reduction air flow 30%
Apnea/Hypopnea index per hr sleep
mild 5-14
moderate>15
severe>30

central: pontomedullary respiratory center problem
absence airflow+abdominal + thoracic movements - on polysomnogram
-heart failure (cheynes stokes)-> get ECHO

vs. obstructive - closed airway
-OSA; incidence the same in post menopausal women and men

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61
Q

sandy orange diapers, UTIs

A

Lesch Nyhan
HPRT mutation

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62
Q

dermal sinus tract

A

terminate in SAH, bone, etc - meningitis

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63
Q

split cord 1 vs 2

A

diastematomyelia
1 - 2 cords 2 sacs
2 - 2 cords 1 sac

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64
Q

curriano triad

A

anterior sacral meningocele + sacral mass + anorectal
-more common in women -urinary Sx

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65
Q

spina bifida occulta vs myelomeningocele

A

spina bifida occulta - folate deficiency - no vertebral body fusion (vs rickets - not vertebral column defect), see lucency in spine
-Depakote (neural tube defects)

myelomeningocele - chiari ii + hydrocephalus

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66
Q

Isaac syndrome

A

Autoimmune disorder - paraneoplastic ; -voltage gated potassium channelopathy
Sx: carpopedal spasm, sweating, myotonia
peripheral nerve hyperexcitability -

-EMG: neuromyotonia ( ping sound , high frequency 150-300 Hz decrementing repetitive discharges) + myokymia - bag of worms continuous muscle twitching

vs. myotonia - 20-100 hz - waxing/waning dive bomber sound

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67
Q

EMG: fibrillation potentials vs complex repetitive discharges vs positive sharp waves

A

denervation: fibrillation physiologic equivalent of positive sharp waves
-Fibrillation - negative component first then positive - denervation (denervation goes down)-“rain on roof” sound
-positive sharp waves - downward deflection waves - denervation

complex repetitive discharge - perfectly repetitive spikes machine like sound
-depolarization of single motor unit spreading to adjacent fibers; high frequency 5-100 Hz

end plate spikes - seashell sound on EMG, low amplitude potentials- up component first then down-normal on needle insertion

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68
Q

Postural orthostatic tachycardia vs neurogenic orthostatic hypotension vs vasovagal

A

POTS-head up increase HR by 30 or more over baseline or >120 per minute
-HR the same
Dx with tilt table
-tx: aerobic exercise, hydration, steroid, midodrine (alpha-1 agonist; causes urinary retention), beta blocker

neurogenic orthostatic hypotension= SBP drops 20 or DBP drops 10 when standing
= MSA, autoParkinson’s (and no increase HR)

Reflex syncope -includes vasovagal defecation/micturition/coughing/swallowing - need EKG
neurocardiogenic=vasovagel
-heart causes exaggerated sympathetic response and brainstem withdrawals sympathetics causing vasodilation

orthostatic hypotension response - general visceral efferent

95% specific to seizure: lateral tongue bite

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69
Q

Phenytoin

A

zero order kinetics = within therapeutic range, PHT half life increases and serum concentrations increase nonlinearly proportional to dose
-nystagmus, ataxia, confusion
-purple glove syndrome - after load, blistering skin

toxocity: gingival hypertrophy, ataxia, diplopia, slurred speech

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70
Q

Zonisamide

A

weak CA inhibitor -> metabolic acidosis
sulfonamide related
blocks T type calcium channels
-can get kidney stones

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71
Q

lamotrigine

A

OCP decrease lamotrigine-estrogen-progestin
-lamotrigine reduces effectiveness of ocps

lamotrigine + VPA = increase SJS risk
-VPA inhibits UGT-glucuronidation->raises LTG

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72
Q

poinsoning

A

arsenic - AIDP, sensory motor neuropathy, garlic breath (GI Sx acutely)
moonshine, pesticides, mining, glass

manganese - parkinsonism - HA, psychosis

GPi hyperintensity (+ CO)
-in liver disease pts, parkinsonism, wedling
Tx: chelation

cyanide - almond, HA, anxiety, vertigo, seizures, encephalopathy, skin flushed
-mining, electroplating, domestic fires fires

mercury - psychosis, gum inflammation, ataxia

organophosphates - cholingergic tox
-miosis vs botulism

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73
Q

tetanus vs tetany

A

toxin decrease interneuron transmission
-disrupts fusion synaptic vesicles

clostridium tetani-toxin targets presynaptic synaptobrevin in spinal anterior horn-> no glycine release->no inhibitory interneurons ->spasm

ANS dysfxn

EMG: high frequency discharges after single stimulus

vs. toxin completing with glycine at postsyn motor neuron - strychnine - drugs, herbal meds

TETANY: acute hypocalcemia - high frequency discharge after stimulus

(high frequency rep stim for botulism)

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74
Q

conduction velocity dependence factors + pacemaker

A

Low/slow CV: low temp, low diameter, low myelination, decreased internode length, tall person

High/fast CV: larger axon, warm, more myelinated, long internode length=faster, short person

Saltatory conduction: myelinated fibers CV 35-75 m/s vs (unmyelinated 0.2-1.5 m/s)
myelin decreases capacitance=less current lost

sodium channel density highest at nodes

pacemaker: can use but limit number electrodes and do not stimulate near device; stimulus shorter than 0.2 and frequency 1Hz

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75
Q

Spinal lesion

A

ependymoma - most common SC tumor- 40 years - intradural intramedullary - CERVICAL-central-bilateral arm weakness and loss of pain + temp

Lymphoma SC - intramedullary - CERVICAL mets

vs syringomyelia - CERVICOTHORACIC, signal in cord - HORNER syndrome b/c descending autonomics
-spares dorsal columns

-cervical spondylosis/hyperextension injuries, syringomyelia, intramedullary tumor-> central cord syndrome

meningioma - THORACIC spine-peak in females 70-80 yrs (in the MIDDLE)

epidural -hematoma - spontaneous in kids - THORACIC spine - T2 hyperintense, T1 iso-hypointense in epidural space, T2 bright in cord
-evacuate in 6 hours= better outcome

Thoracic radiculopathy - truncal radicular pain - diabetes risk
-DDx shingles

vs epidural abscess - LUMBAR, abscess, edema

myxopapillary ependymoma - 30s, M>F, LUMBAR

Neuroectodermal tumors - FILUM terminale, CAUDA equina - heterogenous enhancing - aggressive

vs transverse myelitis - edema; one spinal level

vs astrocytoma - intramedullary lesion, edema

spinal cord hemorrhage - hypointense on GRE, heterogenous signal on T1/T2 (vascular malformation) vs. compressive lesion - bright T2, dark T1

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76
Q

cryptococcus source

A

birds - pigeon poop on mummies

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77
Q

tetany

A

Acute hypocalcemia - high frequency discharge after single stimulus

-chovstek (contraction facial mulcles when tap facial nerve); trousseau - BP cuff inflate -> carpopedal spasm (caused by ischemia)

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78
Q

botulism vs MG vs single fiber

A

botulism: toxin inhibits presynaptic ACh release via cleave SNARE; autonomic dyxfsn
-ACh Presynaptic for the SNS + PNS system; (ACh post synaptic for PNS) = pupillary dilation and low DTRs
- NC: low CMAP with facilitation after exercise or HIGH frequency 20-50 Hz rep stim (like LEMS); jitter abnormal
-fermented seafood
-neonatal MG: intact DTRs

MG: EMG: low frequency stimulation 2-3 Hz, decrease response on rep stim
-no pupillary dilation vs botulism
-MG: post synaptic inhibition (Botulism pre synaptic inhibition)

vs tetanus - continuous motor activity

Single fiber: Jitter - variation in time interval btw 2 AP
-97% sensitivity for ocular MG
99% sensitivity for generalized MG

ACH R Ab: 50% sensitivity ocular MG, 85% sensitivity generalized MG (if seronegative, MUSK positive in 50%)

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79
Q

Hyperhidrosis

A

Primary Hyperhidrosis - ideopathic - axillae palms soles, face, scalp, inguina, etc; bilateral and symmatric
-get skin infections
+sweating during day only
+ family history

Dx: starch iodine test (different powder than thermoregulatory)

Tx: glycopyrrolate - oral -side effects vomiting, constipation
-aluminum salts - antiperspirants
Botox
Endoscopic thoracic sympathectomy - destroys sympathetic ganglia

Regional hyperhidrosis -bilateral axilla, feet, palms, face

secondary hyperhidrosis - night sweats- awake and asleep

syringomyelia - episodic sweating with some posture, autonomic dysreflexia

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80
Q

autonomic testing

A

Test Sympathetic Sudomotor cholinergic
sweat testing-
thermoregulatory testing - POWDER - changes color, shows pattern where someone sweats -
-ex autonomic neuropathy

Quantitative sudomotor axon reflex testing - stimulate nerves innervating sweat glands-postganglionic

Sympathetic cardiovascular adrenergic -*HR, BP with Valsalva, tilt table

HR response to deep breathing - PNS cardiovagal /Valsalva (impaired in diphtheria)

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81
Q

vitamin e deficiency

A

can happen with UC and crohn’s

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82
Q

mononeuritis multiplex

A

asymmetric stepwise progression cranial or peripheral neuropathy
VASCULITIC + systemic symptoms, weight loss
NC/EMG: axonal neuropathy, no demyelination. can biopsy
-vasculitis, autoimmune disease

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83
Q

spinal muscular atrophy

A

SMN1 - AR -mRNA processing gene - (Chr 5)
-loss of SMN1 compensated by SMN2 (more SMN2 the better)

  • abdominen protrusion b/c diaphragm weak
    -minipolymyoclonus - fine tremor of limbs
    -spares facial muscles+ has fasciculations vs congenital myasthenia (ACh R delta subunit mutation) - ptosis, choking, Sx worse end of day

neonatal MG - maternal ACh R Ab transmitted to fetus - hypotonia, intact DTRs

SMA0: prenatal - no motor milestones, resp failure by 1 month - 1 copy SMN2

SMA1: unable to sit (Werdnig-Hoffman) - onset before 6 months
1-2 copies SMN2

II: unable to walk - onset after 6 months-3 copies SMN2

III: able to walk - onset>18 months- 3-4 copies SMN2
EMG-complex repetitive discharge

-SMA4-adult - proximal muscle weakness esp quads - more than 4 copies SMN2

Tx: nusinersin
onasemnogene abeparvovec - SMN1 transgene vector Tx <24 months

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84
Q

sympathomimetics

A

VMAT inhibitor: deplete DA in presyn vesicles -reserpine - tardive

cocaine: inhibits presynpatic DA and NE reuptake - NET DAT CAT - cuts net of fish

amphetamine: presynaptic RELEASE NE, 5HT, DA
(LSD - 5-HT)
-amphetamine toxicity - fluids, benzos, acitvated charcoal

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85
Q

chronic traumatic encephalopathy

A

TAUopathy - hyperphosphorylated tau widspread
-1 perivascular phosphorylated Tau lesion around blood vessel - in sulci of cortex
-multiple concussions -> apathy, aggression, personality change

cerebellar scarring, substantia nigra
-degeneration, atrophy, cavum septum pellucidum —»> temporal lobe, thalamus, hypothalamus, mammillary body atrophy

vs. Prion disease - spongiform degeneration - no inflammatory response, DWI cortical ribbon

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86
Q

Carnitine deficiency disorders -Lipid metabolism beta oxidation

A

Carnitine palmitoyltransferase II deficiency-CPTII gene
- myopathic form: most common - pain and weakness high CK + myoglobinuria after exercise/stress only
-MCC hereditary rhabdomyolysis
-other types neonatal, severe infantile hepatocardiomuscular
-trigger: diazepam, VPA- high ammonia
EMG-normal; biopsy normal
-Dx: elevated long chain acylcarnitines, low free canitine
Tx: carbs, low fat

-CPT1A deficiency - fatty liver pregnancy if carrying infant with CPT1A - carnitine HIGH in serum
carnitine acylcarnitine translocase deficiency - SLC25A20 - low free carnitine
-carnitine biosynthesis disorders
-carnitine transport disorders - primary carnitine deficiency (SLC22A5)

dialysis related carnitine disorder - fatigue, hypotension, anemia - free carnitine cleared more than acyl carnitine in dialysis

secondary carnitine deficiency - dialysis, drugs, inborn errors metabolism (increase excretion acylcarnitines), malnutrition
- less severe vs primary deficiency

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87
Q

Glycogen storage diseases

A

-McArdles myophosphorylase - SECOND WIND clenched fist, difficulty opening hand; contractures, rhabdomyolysis
-2nd wind b/c free fatty acids delivered
Tx: carbs

Lysosomal acid alpha-glucosidase - acid maltase deficiency - Pompe
-adults: proximal muscle weakness, atrophy
Tx - alglucosidase alfa

Glucose-6-phosphatase deficiency - Von Gierke - hepatomegaly, hypoglycemia, growth retardation; high lactate, uric acid

Galactose-1-P-uridyltransferase - galactosemia - cataracts, failure to thrive, aminoaciduria

liver/muscle debranching - Cori - hypoglycemia, cardiomegaly, hepatomegaly

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88
Q

transient global amnesia

A

can be triggered by coitus or emotional/physical stress

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89
Q

Parkinson’s pathology and genes

A

eosinophilic cytoplasmic inclusions = Lewy bodies - pale halo

-dopaminergic denervation striatum
-pallor substantia nigra
-pallor locus coeruleus

Late onset; LRRK2
early onset - DJ1 - AR, PINK1 - AR
PARK1 - AD
PARK2 - AR

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90
Q

neurotransmitters basal forebrain, histamine

A

basal forebrain - ACh,
substantia innominata
nuc accumbens

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91
Q

myoclonus Tx

A

Keppra, VPA, benzos (clonazepam, midazolam), topamax

worsen myoclonus: carbamazepine, phenytoin, gabapentin, lamotrigine

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92
Q

Sydenham chorea

A

Tx - penicillin + DA antagonist
-fluphenazine

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93
Q

restless leg syndrome

A

augmentation: DA meds cause Sx early in day, more severe, other body parts (can happen after 1 month)

kids - check iron panel, can have comorbid ADHD, periodic limb movements of sleep

Tx iron deficiency ; first line gabapentin/pregabalin
Tx OSA improves Sx

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94
Q

Histopathology

A

Target fibers - succinate dehydrogenase staining - denervation

Cowdry A inclusions - herpes

Plasmodium - hemozoin - malarial pigment
toxoplasmosis - bradyzoites

schwanommas - Antoni A + B cells

metastatic adenocarcinoma - simple and tubular glandular cells, rim enhancing cystic mass

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95
Q

factitious vs malingering

A

factitious - want to be a patient , no secondary gain

malingering - needs secondary gain like money or disability or drugs

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96
Q

RNS DBS treatments

A

RNS: multifocal or focal and not a surgical candidate
-gives stimulus at seizure onset, closed loop system (vs DBS, VNS - open loop)

-side effects less common in RNS vs. VNS+DBS b/c give focal stimulus

DBS: 1st- approved for essential tremor - Ventral intermediate nuc thalamus
2nd- dystonia - GPi
3rd OCD
4th focal onset epilepsy - ant thalamus - 2008
5th - MDD treatment resistant - most recent

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97
Q

Brain tumors kids

A

INFRATENTORIAL
Pilocytic astrocytoma - Cerebellar Hemispheres (medulloblastoma is midline and roof of 4th ventricle
-cyst with enhancing mural nodule
-if NF1 -> in optic tract
-presenting Sx MASS EFFECT
-bipolar neoplastic cells with hair like processes (Rosenthal fibers)+eosinophilic granular bodies (eosinophilic hyaline masses)
-fusion K-BRAF protein
Tx: surgery, BRAF inhibitor

Ependymoma - 4th ventricle FLOOR-perivascular pseudo rosettes

Medulloblastoma - MIDLINE vermis or ROOF fourth ventricle - Homer Wright Rosettes
(DROP things from the ROOF)
-age 2-6

4 subgroups: WNT, SHH, group 3, group 4
1. WNT - BEST prognosis - CTNNB1 mutation (kids, adults)
2. SHH - PTCH1 gene, MYCN, GLI2 - intermediate - infants, adults
3. group 3- worst prognosis - MYC amplification - infants, children
4. group 4- CKD6 (MYCN)- intermediate prognosis

-Homer-Wright pseudorosettes
-get spinal imaging b/c drop metastasis
-Turcot syndrome - gliomas, colon polyls, adenocarcinoma, medulloblastoma - APC

SEGA - 3rd or lateral ventricles
-TSC only
Histo: -calcifications
-candle gutterings

SUPRATENTORIAL
Pleomorphic Xanthoastrocytoma - temporal lobes
-histo: intercellular reticulin deposit

Ganglioma - temporal lobe

hemangiopericytomas - staghorn vasculature

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98
Q

Supratentorial tumors

A

SATCHMOE - sarcoid, aneyrusm/adenoma, teratoma, craniopharyngioma, hypothalamic glioma, mets, opic nerve glioma, epidermoid

  1. Ganglioglioma - temporal - cyst with mural nodule - BRAFV600E
    -neuronal + glial on histo
  2. Embryonal tumor with multilayered rosettes - pseudostratified epithelium with central clear lumen - heterogenous contrast enhancement, diffusion restriction
    -frontal, parietotemporal
    -ages <5
    -C19MC amplification on chromosome 19
    if no C19M classification but has pseudostratefied neuroepithelium -> medulloepithelioma

Dysembryoplastic neuroepithelial tumor - DNET - no contrast enhancement or diffusion restriction - glioneurla nodules, foci cortical dysplasia

Pleomorphic Xanthroastrocytoma

Craniopharyngioma - Rathke pouch - palisading epithelial cells; cystic component
-calcifications
-papillary craniopharyngioma - non keratinizing squamous epithelium + fibrovascular core

vs pituitary adenoma - hypercellular with same cell type

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99
Q

cyst with mural nodule DDx

A

pleomorphic xanthroastrocytoma

ganglioma

pilocytic astrocytoma

hemangioblastoma - cyst usually does not enhance

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100
Q

radiation plexopathy

A

MRI - enhancement - tram track appearance
FDG-PET - no uptake vs tumor
EMG-myokymia (worm like movements under skin) - sound like soldiers marching + fasiculations
not painful vs. tumor infiltrative plexus

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101
Q

confusional migraine

A

Rare Pediatric migraine
speech slurred, vomited, disoriented, agitated x 6-24 hours
Tx. topiramate (blocks VG NA and Ca channels + blocks CA, enhances Gaba

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102
Q

Moya moya

A

vasculopathy of narrowed distal ICA + branches
watershed strokes
puff of smoke

-limb shaking, chorea - disrupt BG circuit

-no inflammation or athero on biopsy -> not inflammatory
-intimal hyperplasia, interruption internal elastic lamina, proliferation sm muscle

Tx - ASA, no AC

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103
Q

craniosynostosis

A

one sutures or more closes early

primary - ossification problem
if 2 sutures close early -> increased ICP
-sattigal suture - scaphocephaly; coronal - anterior plagiocephaly - bilateral coronal - brachycephaly
lambdoid - posterior plagiocephaly; metopic - trigonocephaly

-Crouzon - fibroblast growth factor-2 gene. bicoronal craniosynostosis - brachicephaly, proptosis, midface bony hypoplasia - hands normal vs Aperts, Pfieffer

secondary -most common- small brain growth-> early fusion -MICROcephaly-

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104
Q

NMO, ADEM

A

BL optic neuritis
aquaporin 4 channel - astrocytic foot processes, gray matter spinal cord

acute Tx - IVIG, steroids, PLEX

Interferon Beta can worsen disease b/c -B cell mediated disease (INF B T cell)
natalizumab, fingolimod worsens disease

reduce relapse - rituximab, azathioprine, eculizumab

For:
ADAM Tx - steroids 1st line, then plasma exchange, IVIG

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105
Q

Cruciate paralysis

A

cervicomedullary junction

brachial diplegia -
bilateral arm weakness, spares legs SPARES SENSation + dysarthria/dysphagia (lower CN)

-CST with arms decussates one segment higher than legs, more anterior, more susceptible to injury
-vs. C spine - arms and legs and sensation affected

-odontoid fracture, atlantoaxial

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106
Q

B12 deficiency

A

no Methylmanonyl CoA to succinyl coA-> high MMA

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107
Q

dopamine precursors

A

phenylalanine, tyrosine

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108
Q

infantile spasm

A

ACTH first line
genes: STXBP1

vigabatrin if TSC - causes VF deficits
-inhibits GABA-T -> increases GABA

hypsarrythmia - best seen during quiet sleep - need 24 hrs EEG

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109
Q

ethosuxamide side effects

A

N, V, sleep problems, hyperactivity

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110
Q

Migraine neurostimulation + biofeedback

A

Vagal nerve stimulation - cluster + migraine

DBS not approved for cluster

migraine abortive + preventative: transcranial magnet-nTMS ,external trigeminal stimulation; vagal nerve stim, occipital stim

Cefaly - transcutaneous supraorbital neurostimulation - episodic migraine - forehead device - use once/day x 20 min - stimulate supraorbital N (trigeminal N branch)

Biofeedback: migraine prevention - control physical response to stress- body functions not under conscious control - (not Emotional responses)

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111
Q

essential vs physiologic tremor

A

physiologic = able to perform tasks
-symmetric
-risk with SSRI

essential tremor - asymmetric, worse with action
-don’t need to check serum copper

-postural tremor - worse in wing beating vs arms out

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112
Q

concussion

A

prognostic indicator: more severe Sx within first few days
-severity of concussion does not correlate with post concussive syndrome risk

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113
Q

Dx bipolar I

A

Manic episodes lasts 7 days
-increase in goal directed activities
-don’t need depression in bipolar I

(bipolar II-hypomanic + MDD)

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114
Q

cervical myelopathy

A
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115
Q

ADHD

A

if >6 years: stimulants ; no increase dependence

if epilepsy - low dose methylphenidate

-if tic disorder/ASD aggress - alpha 2 agonists - clonidine - better if comorbidity

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116
Q

Perilymphatic fistuala

A

perilymph fluid leads from inner to middle ear
-vertigo, disequilibrium
-sensorineurla hearing loss, left beating nystagmus

Tulio phenomenon: disequilibrium triggered by loud noise or valsalva

cause: congenital abnormality temporal bone or trauma

vs Meniere - dilation endolymph - Sx better with salt restriction; diuresis to decrease endolymph
Tx - endolymphatic hydrops

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117
Q

refractory epilepsy neonates

A

pyridoxine dependent epilepsy
-deficiency alpha-aminoadipic semialdehyde dehydrogenase - antiquitin
-clonic seizures

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118
Q

Phantom limb pain vs residual limb pain

A

phantom limb: more common upper limb
peripheral + CNS hypersensitization

vs residual limb pain: associated with underlying cause

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119
Q

CVST w/u

A

CTH THEN MRI MRV before LP
-get bHCG

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120
Q

Foster Kennedy Syndrome

A

Mass compressing optic nerve, ipsilateral optic pallor/atrophy, contralateral papilledema, anosmia
ex: sphenoid wing meningioma

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121
Q

Benign idiopathic neonatal convulsions and benign familial neonatal seizures vs benign familial infantile seizures

A
  1. Benign idiopathic neonatal convulsion
    fifth day fits, seizure 4-6 days
    -variant theta rhythm on EEG
    -clonic seizures
    -KCNQ2 - deletion (loss of function of 1 gene = 50% function)

Vs mis sense mutation KCNQ2 - gets incorporated into channel and even less function (less than 50%) - get DEE not fifth day fits

  1. Benign familial neonatal seizures
    KCNQ2, KCNQ3 gene, AUTOSOMAL DOMINANT
    -EEG NORMAL, MRI normal, normal growth/dvelopment

Tx: no tx or oxcarb, carbamazepine

  1. benign familial infantile seizures; SCAN2A, SCAN8A - Autosomal dominant
    -start at 6 months and seizures end at 2 years
    -association with adult paroxysmal kinesigenic dyskinesia in adolescence-PRRT2 gene - proline rich transmembrane protein 2 gene - Chr 16 p

febrile seizures-GEFS+. SCN1A

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122
Q

Hypothalamus

A

lateral - orexin

suprachiasmatic - insomnia, sleep wake

arcuate - > secretes stimulating and inhibitory releasing hormones - tuberohypophyseal tract

paraventricular nucleus - autonomic fibers

anterior hypothalamus - regulates hyperthermia
lesion = get hyperthermia

posterior - regulates hypothermia
lesion = get hypothermia

paraventricular (magnocellular), supraoptic - ADH, oxytocin - [subforniceal organ senses volume] ->secreted from posterior pituitary/neurohypophysis from neuroectoderm

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123
Q

Panayiotopoulos syndrome vs cyclic vomiting syndrome vs SELECTs

A

occipital epilepsy, see elemental shapes + nausea, vomiting, tachycardia, tachypnea

4-8 years
EEG: high voltage occipital spikes

Don’t need Tx but use carbamazepine

vs Gastaut-type epilepsy - last onset childhood occipital - usual present ~ 8 years, + post ictal migraine

SELECTS - facial parethesias, jerking face, hypersalivation- centrotemporal spikes - age 4-11 yrs

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124
Q

Radial neuropathies

A

Wartenberg - superficial radial nerve-sensory - compression watch/handcuffs - parthesias dorsolateral hand
-motor intact

posterior interosseus neuropathy - weakness ulnar wrist extension; radial wrist extension spared, sensory spare

vs. ischemic monomelic neuropathy - AV shunt placement; weakness/sense loss in multiple distributions, radial pulse still palpable

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125
Q

median neuropathies

A

pronator teres syndrome - median nerve - pain in forearm, sensory loss over thenar eminence (intact in Carpal tunnel);
[supinator is radial]
anterior interossei - weakness, sensory spared - DIPs 2 and 3, flexor pollicus LONGUS, pronator quadratus -“OK sign”
-neuralgic amyotrophy

CARPAL TUNNEL - flexor retinaculum (NC - prolonged distal latency + slow CV b/c focal demyelination( if axonal damage, reduces amplitude)
-tinel/phanel/hand raise test
-spares thenar eminence
-palmar mixed study significant different median + ulnar 0.4 ms
-combined sensory index of 1 or more - maximize sensitivity without reducing specificity -different tests anti-orthodromic

median hand - 1/2 LOAF-LBS (flexor pollicis longus+flexor pollicis brevis superficial head)
- lateral 2 lumbricals
opponens pollicis brevis, ABductor pollicis brevis APB, flexor pollicis longus (OK sign)+flexor policis brevis superficial head

NOT adductor pollicis (ulnar); flexor pollicis brevis deep head- ulnar
abductor pollicis longus - radial

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126
Q

Hartnup disease

A

SCL6A19 - chr 5
diarrhea, red flaky skin, agitation ~Niacin deficiency, sun induced rash

-AR, defective amino acid transporter - increase excretion nonpolar amino acids=tryptophan + phenylalanine in urine
-no tryptophan absoroption -> niacin precursor

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127
Q

femoral nerve

A

motor: hip flexion, knee extension
anterior femoral - sartorius, pectinus

posterior femoral - rectus femoris, vastus

-above inguinal ligament - ilacus weakness
below inguinal ligament - spared (iliopsoas) - hip flexion

sensory above knee: medial thigh
sensory below knee - saphenous - medial lower leg, foot

128
Q

lateral femoral cutaneous

A

sensory only - anteriolateral thigh
from L2-L3 directly NOT from femoral
-no w/u if patient presents with this only

129
Q

post stroke fatigue vs depression

A

fatigue: can use adrenergic/dopaminergic - modafinil
-depression only in late stages
-no associated with sex or age

depression: Beck depression inventory quantitative over time
post-stroke depression + DM2 - pioglitazone effective, unknown MOA
-SSRIs: risk spontaneous ICH
-Barthel index - ADLs+mobility, sensitive to psychological factors on recovery

130
Q

OCD Tx kids

A

SSRI first line; clomipramine (TCA)
Dx - compulsions or obsessions or both

131
Q

motor evoked potential

A

CONTRALATERAL primary motor cortex
-CST pathway

132
Q

glioblastoma multiforme + anaplastic astrocytoma

A

Choline peaks > NAA peaks in gliomas (reversal of Hunter peak)
Cho>Cr>NAA

GBM: choline> lactate > NAA
-lactate high in areas of necrosis

normal = NAA peak highest

NAA = neuronal integrity
choline - cell turnover
creatine - energy stores (depleted in tumor)
-lipid peak = cell breakdown

anaplastic astrocytoma - nuclear atypia, pleomorphism, increase cellularity

133
Q

Trigeminal nerve anatomy - inferior alveolar

A

-Ophthalmic-
nasociliary - nose senes

-Mandibular-
1. inferior alveolar - branch CN V3 mandibular
-dental nerve block
2. mental-branch of mandibular - chin, lower lip
3. lingual - anterior 2/3 tongue
4. buccal - (that) inner cheek

134
Q

developmental venous anomalies

A

caput medusae

135
Q

nucleus ambiguus vs solitarius

A

ambiguus - CN IX, X - motor only - larynx and pharynx muscles - special visceral efferents pharyngeal arch; and CN VII - stylopharyngeus

solitarius - a Singular Sensation - taste sensation - 7 (chorda tympani anterior 2/3), 9, 10 (epiglottis)
-autonomic nuclei-solitary nucleus
-general visceral afferents: carotid body/sinus - CN 9; SA node/atria CN X

136
Q

brain death examination

A

no spontaneous breathing/chest wall movement -> apnea test
-if fail apnea test AKA have spontaneous breathing, not consistent with brain death

-need SBP >100, temperature >36

HIE: 24 hours or 24 hr after rewarm
-don’t need neurologist

ancillary: transcranial doppler - small systolic peaks, no diastolic flow
-absent BL N19-P22 cortical potentials with median N stim
-brainstem auditory evoked potentials: absence brainstem responses waves III-V with preserved cochlear response wave I
-absent blood flow on MRA
-EEG (old now) - 10 nin, 2 microV

137
Q

autoimmune autonomic impairment

A

ganglionic nicotinic ACh R
-severe autonomic Sx, dilated pupils
-associated with SCLS paraneoplastic
-subacute <3 months Sx
Tx: plasma exchange, IVIG, but poor prognosis

vs HSAN - sensory deficit
HSAN3- Riley Day- AR

138
Q

Waardenburg syndrome DDx

A

Waardenburg - AUTOSOMAL DOMINANT - warlock with a white forelock, deafness
-defect NCC migration

vs Legius (like NF1 but no Lisch nodules) vs

Incontinentia pigmenti -, vesicular lesions/pustules

vs Noonan with multiple lentigines (brown spots)- LEOPARD - electrocardiogram conduction defects, ocular hypotelorism, pulmonic stenosis, abnormal genitalia, growth retardation, deafness

139
Q

axillary vs suprascapular

A

POSTERIOR cord
-Axillary N - C5-C6 –delt, teres minor (external rotation); triceps long head
-sensation shoulder
-anterior shoulder dislocation

-TERES MAJOR-lower subscapular nerve- C5, C6, and C7 nerve roots- internal rotation
-SUBSCAPULARIS - superior+inferior subscapular N

-thoracodorsal - latissimus dorsi - shoulder adduction
__________________________________________________
UPPER trunk
suprascapular: off upper/superior trunk plexus
-scapular fracture, ganglion cysts, overuse, Parsonage turner/neuralgic amyotrophy/brachial neuritis
-supraspinatus - first 15 degree shoulder abduction with internal rotation (empty can test) + infraspinatus - external rotation

140
Q

Burst suppression DDx

A

Otohara - tonic seizures - see abnormality on MRI

infantile onset epileptic encephalopathy - STXBP1 - syntaxin binding protein
-infantile spasms
hypasarrhythmia, burst suppression

141
Q

Hyperexplexia

A

GLRA1 - Chr5
-inhibitory glycine R

hyperkinetic movement disorder - nocturnal myoclonus, exaggerated startle, stiffness , NO seizures

142
Q

AC reversal

A

warfarin - FFP, vit K (warfarin in rat poison)

andexanet alfa - DOACS

heparin - protamine supfate

idarucizumab - dabigatran

143
Q

Medial cord, Ulnar N. vs C8 radic

A

Ulnar aspect arm: medial
medial cord C8-T1: medial cutaneous nerve of arm + forearm both come off medial cord

C8 radic - (exit C8/T1 marking where nerves exit below vertebrae) normal SNAP
medial forearm sensory loss

lower trunk - abnormal sensory response

144
Q

musculocutaneous N, lateral cord; arm sensation

A

lateral cord gives off lateral pectoral nerve
-musculocutaneous N -lateral antebrachial nerve branch-lateral forearm ) -
lateral arm sense - inferior lateral cutaneous nerve - radial N branch
medial arm sense is medial antebrachial off medial cord
-median nerve with medial + lateral cord

145
Q

Landau Kleffner

A

acquired epileptic aphasia

ESES-electrical status epilepticus sleep continuous BL centrotemporal spike wave discharges in nonREM sleep

Tx-VPA, diazepam, steroids

(vs. Benign rolandic epilepsy - unilateral centrotemp discharges)

146
Q

vagal nerve stimulation

A

indication: drop attacks (also callosotomy for atonic seizures),
Rasmussen encephalitis, Sturge Weber, lesional epilepsy, MTS, myoclonic seizures
-goal to decrease seizures by 50%
-Treats depression, (migraine, cluster HA)

-takes 1 yrs to work,
-Side effects: hoarseness/voice changes in 2/3 pts (transient); cough

ck battery q6 months

147
Q

Frey Syndrome

A

auriculotemporal syndrome/gustatory sweating
sweat, flush cheek when eating in auriculotemporal nerve distribution

parotid injury -> no sympathetic cholinergic fxn for sweating -> aberrant regeneration PARAsympathetic fibers
-> paradoxical sweating with parasympathic stimuli - chewing

148
Q

Primary neurulation

A

Day 24-26
-closure anterior neuropore -> lamina terminalis -> anterior wall 3rd ventricle

-anencephaly if fail

149
Q

duchenne, becker muscular dystrophy

A

XLINKED RECESSIVE
Sx: no reflexes

Tx: steroids first line; deflazacort
etiplersen - ASO antisense - oligonucleotide - restores dystrophin if mutation in exon 51 (~14% pts)

idebenone - co Q10 analong - RESpiratory function (edaravone - ALS)

150
Q

dural AVF vs AVM vs DVA

A

dAVF - no nidus - direct artery to venous sinus
-acquired

AVM - nidus - flow voids T2 dark
-abnormal connection btw arteries + veins

DVA: irregular arrangement small veins draining into larger vein - “spokes on a wheel” - not danverous

cavernoma - collection of abnormal dilated blood vessels - popcorn - usually asymptomatic

151
Q

carotid artery dissection, Horner’s

A

-resolution in 3-6 months
-usually no repeat recurrence unless risk factors
-HA improves after 1 week

-partial horners, no anhidrosis if ICA dissection b/c superior cervical ganglion at carotid bifurcation

-oculosympathic pathway (pupil constriction)-superior cervical ganglion: ascend via ICA to cav sinus, pupil dilator, Muller muscles

-no response to ciliospinal reflex: painful stimulus to upper cervical/facial area -no expected pupil dilation-

152
Q

Hemiconvulsion-hemiplegia epilepsy syndrome

A

prolonged focal motor seizure during febrile illness
-acute cytotoxic edema on MRI

vs. Rasmussems - focal epilepsy, prodrome mild hemiparesis, isolated seizures, epilepsia partialis continua

153
Q

Bruxism

A

-serotonin antidpressants risk of bruxism
-anxiety
-incidence peaks childhood
Tx: can use clonidine, clonazepam but not that effective

154
Q

heritable dystonias

A

Segawa - Dopa responsive dystonia - GCH1 (GTP cyclohydrolase) deficiency - AD -
(“pregnancy HCG movement disorder good prognosis”)
-tyrosin hydroxylase deficiency
-foot dystonia in kid, diurnal fluctuations, parkinsonism later

-focal to generalized: more likely if young kid first in leg than in arm

-geste antagonist in 60%

Generalized dystonia - Torsin gene DYT-TOR1A - AD
-not DA rseponsive
-trihexyphenidyl, tetrabenazine

Paroxysmal kinesigenic dyskinesia - episodic chorea, dystonia from voluntary movement - PRRT2 gene - proline rich transmembrane

rapid onset dystonia-parkinsonism - ATP1A3 gene - segmental dystonia worsening over hrs-days after emotional/physical trigger

(ATP1A2- familial hemiplegic migraine 2 without cerebellar Sx)

Spasmodic dysphonia - “whispering dystonia” - TUBB4 - characteristic facies, body habitus

155
Q

DDx infant hypotonia

A

Leigh syndrome - subacute necrotizing encephalomyelopathy
-hyperreflexia
-microcephaly
-no tracking ->cortical visual processing problem
-still antigravity in limbs
-ophthalmoplegia
-mitochondrial disease can present at birth (vs. Dravet - 6 months)

vs spinal musuclar atrophy - DTRs down, no microcephaly
vs myotonic dystrophy - weaker, DTRs absent

156
Q

myasthenia gravis drugs worsen

A

CCB
fluroquinolones, Bblockers, aminoglycosides, clindamycin, vancomycin, procainamide, quinidine, anti-PD1 monoclonal Ab (nivolumab), Botox, penicillamine

157
Q

special visceral effect vs general somatic efferent vs general visceral efferent

A

special visceral efferent - CN V, CN VII, pharyngeal arch derived muscles mastication, facial expression, IV, X palate pharynx, larynx

general somatic efferent - somatic mesoderm - voluntary muscles - intrinsic + extrinsic tongue muscles - hypoglossal N - (from somites not pharyngeal branches)

general visceral efferents -autonomic muscles - orthostatic hypotension response

158
Q

episodic ataxias

A

Autosomal dominant
type 1 - facial myokymia - KCNA1
-acetazolamide, carbamazepine

“ Eat 1 KFC and wipe grease off my face [facial myokymia]”

EAT2 - CACNA1A - MOST COMMON - brainstem symptoms - triggers stress, EtOH - nystagmus, dysarthria
acetazolamide (eat 2 much calcium and choke)

159
Q

autonomic dysreflexia

A

bowel/bladder stimulus ->sympathetic response ->vasoconstriction -> HTN -> HR slowed
-if T6 and above

160
Q

delayed sleep wake phase disorder

A

10% insomnias, FHx
-2 hours before falling asleep - melatonin secreted later

short wavelength blue light bad ?

161
Q

hereditary aneurysms vs AVM vs hemangioblastoma

A

hemangiomblastoma - VHL - RCC + phenochromocytoma

aneurysms, dissection - Marfans

AVM - Osler Weber Rendu - hereditary hemorrhagic telangiectasia - AD

162
Q

oculopharyngeal muscular dystrophy

A

GCN/GCG expansion, PABPN1, Chr 14
French Canadian
-ptosis, dysarthria, EOM, facial weakness

163
Q

serotonin syndrome vs NMS vs sympathomimetic vs anticholinergic

A

serotonin syndrome: HYPEReflexia, increased bowel signs, mydriasis low urine OP, coagulopathy
Tx - charcoal if within hrs; cyproheptadine (5-HT antagonist)

NMS - lead pipe rigidity, normal or decreased reflexes; mydriasis or normal pupils; normal bowel sounds - antipsychotics
-cause: central, peripheral acute DA block- risk in men who recently increased antipsychotic
-Tx - benzos, bromocriptine, dantrolene (ryanodine receptor antagonist)

sympathomimetic: withdrawal or abuse -reflexes NORMAL, normal bowel sounds; mydriasis
- Tx charcoal, whole bowel irrigation

anticholinergic - dry, mydriasis

malignant hypothermia - normal pupils , normal bowel sounds, hyPOreflexia

164
Q

neurosarcoid

A

thick patchy leptomeningeal enhancement

-hypothalamic inflammation ->neuroendocrine dysfxn -> polyuria, polydipsia, sleep, appetite, temp, libido
-pituitary inflammation
-inflammatory myelopathy
-peripheral neuropathy-small fiber, mononeuritis multiplex, aspetic meningitis
-optic neuropathy

165
Q

XL adrenoleukodystrophy

A

ABCD1 - LOF ALD protein
-earliest signs behavior changes + MRI involvement -> stem cell transplant (even if older boys)
-no stem cell transplant if no MRI involvement b/c not all have CNS disease

-posterior ventricular white matter disease

Lorenzo oil - lowers very long chain FA concentrations - not effective

166
Q

Reflex epilepsy

A

reflex = provoked by specific stimulus -
-eye closure sensitivity

  1. photosensitive epilepsy - sunflower
    -Most common
  2. orofacial reflex myoclonic - talking/reading
  3. praxis induction - motor tasks - muscle jerks
  4. musicogenic epilepsy - music provokes seizures

Mostly GTCs
tx - VPA, LTG, benzo

167
Q

lumbar plexopathy vs femoral neuropathy

A

lumbar plexopathy: weak thigh adductors, absent saphenous sensory
-retroperitoneal hemorrhage
normal paraspinals

Femoral neuropathy: thigh adductors spared

radiculopathy: abnormal paraspinal response;
-neck pain + loss reflexes
-sensory noral

L2-L3 - lateral femoral cutaneous nerve
S1-S2 - piriformis

168
Q

CME vs self-assessment

A

Self assessment/self-audit : defined quality metrics - to stay up to date on standard of care
-compare your practice to standard of care on multiple quality metrics

CME: limited applicability to your practice

board exam - no regular ongoing feedback

169
Q

SSRI side effects

A

RCVS - SSRI, SNRI adjustments

bruxism

170
Q

Ataxias with vitamin e deficiency

A

Ataxia with vit E deficiency - low alpha-tocopherol -
-diet or AR mutation TTPA (vit E to liver)
Tx: ORAL vit E (not IV b/c good absorption)

Abetalipoproteinemia - AR - MTTP
-acanthocytosis, multiple DEAK deficiencies
-low total cholesterol, low LDL

Tangier - ABCA1 casette protein -
-low total cholesterol, low HDL
A healthy tangerine with low cholesterol, high TGs; orange tonsils
-can mimic syringomeylia
histo: foamy macrophages

vs, pyruvate dehydrogenase deficiency - episodic ataxia, dystonia, lactic acidosis - infancy to childhood

171
Q

tic paralysis

A

Dermacentor ticks
can develop over days
-inhibition ACh release after tick feeding for days

171
Q

Midline defects

A

holoprosencephaly - failure prosencephalon divide

Schizencephaly - migration/organization-1st trimester problem -lined with gray matter
(porencephaly - line with white matter)

hydranencephaly - acquired infectious/vascular insult last 1st/2nd trimester- hemisphere replaced with CSF, only brainstem + cerebellum left

Polymicrogyria - 2nd trimester - problem organization
focal cortical dysplasia - organization problem

172
Q

retinoblastoma, Li Fraumeni chromosome

A

retinoblastoma - Chr 13 - can have stabismus, glaucoma, nystagmus, proptosis, anisocoria (Not corneal arcus)
Li Fraumeni - Chr 17

172
Q

Kyphoplasty

A

Tx pathologic vertebral body fractures, compression >50% (mets to spine)

Balloon assisted vertebral body height restoration + cement augmentation

173
Q

EOM palsies anatomy, trochlear nerve vs nucleus

A

Superior orbital fissure - unilateral CN IV, III, VI

CN IV: BL trochlear N palsy at superior medullary velum - decussates in medullary velum as exits from dorsal midbrain
-innervates superior oblique
-alternating hypertropia = R hypertropia with L gaze, etc

-unilateral trocholear nerve palsy (SO-no depression, intorsion, adduction eye): CONTRALATERAL TILT of head to compensate
-worse in contralateral gaze, ipsilateral head tilt
(ipsilateral CN IV nerve or contralateral CN IV nucleus palsy)

Petrous apex: Gradenigo syndrome - Cn VI palsy, facial pain, CN V1 sensory loss

ponto-medullary junction - CN VI palsy

174
Q

nervus intermedius neuralgia

A

stabbing pain deep in ear CN VII - intermediate nerve of Wrisberg branches off facial nerve at geniculate ganglion (same as Ramsay Hunt)
Tx: carbamazepine

175
Q

Friedrich’s ataxia vs CMT, DDx

A

Friedrich’s ataxia - loss sensory potentials, normal motor CV
-DIABETES - unique to FA
-hunchback, levoscoliosis, pes cavus

CMT 1A - slow CV
EM: no conduction block like CIDP (conduction block=no response on NC stim at proximal site and response at distal site)
CMT1B-myelin protein zero (MPZ) mutation

CMT 2: low CMAP and sensory amplitudes
-reduction myelinated fibers, onion bulbs from Schwann cell proliferation

CMT2-MFN2 mitochondrial fusion protein mitofusin 2 (like MFM=adult=axonal)
X linked CMTX-GJB1

giant axonal neuropathy - curled hair, walk insides feet, childhood distal sensory/motor neuropathy
-mutations in gigaxonin

176
Q

INO - what side is lesion compared to nystagmus?
One and a half vs INO vs PPRF

A

Eye that can abduct with nystagmus: contralateral to lesion side
Eye that cannot adduct: ipsilateral to lesion side

One and a half syndrome: pons tegmentum - PPRF (no eye movement when looking to lesion side), MLF

just PPRF lesion: can’t look ipsilateral to lesion with either eye + coarse end gaze nystagmus looking contralateral

177
Q

Coccidioidomycoses

A

-subacute meningitis, southwest US
Tx - fluconazole even for meningitis
Dx: CSF coccidioides antigen + PCR testing

178
Q

Alcoholic hallucinosis vs delirium tremens

A

Seizures: 6-48 hours

[Hallucinosis: 12-48 hours - visual+tactile hallucinations, preserved orientation before DTs]

DT: 48-72 hours - confusion + hallucinations

179
Q

vertebrobasilar ischemia

A

orthostatic hypotension with new BP meds
-vertigo, drop attacks, perioral numbness
->compression vertebral artery - bone spurs
-comparative BP measures standing/sitting and auscultation neck-> to check for subclavian steal

180
Q

percentage sleep stage by age

A

N2>REM>N3 slow wave
N2 is 50% of sleep for all ages
-REM suppressed by meds

younger: more N3, more REM, less N2
older: more N2, less N3, less REM

narcolepsy - REM early in sleep

181
Q

prosopagnosia, Balint

A

bilateral fusiform gyri (vs temporal-occipital - difficulty with object recognition)

Balint - bilateral parieto-occipital lobes

medial occipital - Anton syndrome - cortical blindness - NO optokinetic response

182
Q

atonic seizures exacerbate, Tx

A

exacerbate: carbamazepine, phenytoin

rufinamide just for LGS- risk short QT syndrome

183
Q

Takotsubo

A

ICH, stroke, SAH
-can have ballooning outside the apex
-normal cardiac cath

184
Q

Kennedy disease X linked spinobulbar muscular atrophy weakness pattern vs ALS

A

proximal > distal weakness, LMN signs only
-Diabetes
X linked CAG repeat in androgen R gene
-perioral/facial twitching (may not give gynecomastia)
EMG: diffusely large motor units (reinnervation)
absent sensory responses

vs MMN-GM1 Ab-pure motor problems

185
Q

ASMs avoid in mitochondrial disease

A

carbamazepine, phenobarbital, VPA, phenytoin

186
Q

Diphtheria mechanism arrhythmia

A

cardiac vagal denervation -> parasympathetic dysfxn
abnormal bradycardia/valsalva response

187
Q

absence epilepsy

A

20 sec or less, no postictal confusion

188
Q

SAH management

A

Noncommunicating hydrocephalus -> EVD (lumbar drain for communicating hydrocephalus)

no hyperventilation b/c vasoconstriction -> vasospasm

vasospasm peak 5-7 days

189
Q

Coital HA Tx

A

indomethacin

190
Q

medial, lateral pectoral nerve

A

medial pectoral - off medial cord C7,C8 / C8/T1
-medial brachial, medial antebrachial
pectoralis minor and the sternocostal head of pectoralis major

lateral -off lateral cord

191
Q

oculocerebrorenal syndrome / Lowe

A

OCRL1 gene
X linked
-cataracts, glaucoma
-renal failure
-areflexia, hypotonia

192
Q

kluver-bucy vs witzelsucht

A

Witzelsucht: inappropriate jocularity - OFC also
(OFC also -antisocial)

amgydala - Kluver Bucy

gegenhalten - paratonia
ganser syndrome - dissociative disorder

193
Q

cervical myelopathy Sx + indicators poor prognosis

A

Symptoms > 18 months
Age, female
urinary symptoms
Asian -post. long ligament ossification

fasciculations only in arms (C5-C7) vs ALS
-EMG with changes only in UE

[primary lateral sclerosis - only UMN]

flail arm ALS - brachial amyotrophic diplegia - proximal arm, asymmetric, spreads distally

cervical roots exit above, thoracic roots exit below

194
Q

spinocerebellar ataxia

A

SCA1 - CAG repeat ATXN1 gene chromosome 6
-SCA3: Machado joseph- most common - CAG ATXN1 gene
SCA 7 - teenage, early adult - retinopathy

autosomal dominant

195
Q

ulnar N, Froment sign

A

Proximal to Guyon Canal:
forearm: flexor carpi ulnaris, flexor digitorum profundus 4, 5 (flexes DIP);
-flexor carpi ulnaris
-palmar cutaneous nerve - sensation over hypothenar eminence

Distal Guyon Canal:
-4th + 5th lumbricals - flex at MCP
-adductor pollicis - thumb ADDuction weakness
Isolated loss of the 5th digit ulnar SNAPs could be seen with an injury distal to Guyon’s canal and proximal to the 5th digit. A
at guyon canal - Froment sign - flex thumb to compensate for adductor pollicis weakness

-dorsal, palmar interossei- finger adduction/abduction at MCP
- flexor pollicis brevis deep head - thumb flexion at MCP

mid-palm injury: isolated involvement FDI CMAP as this would spare the branches to the hypothenar eminence and the digits.

hypothenar muscles (abductor digiti minimi)

196
Q

Tx HTN acute stroke
Risk with home med and TPA?

A

NiCARDipine CCB, clevidipine, labetalol

ACEi increase bradykinin -> increases risk orolingual angioedema bc TPA increases bradykinin via thrombolysis

197
Q

polymicrogyria, lissencephaly genes

A

LIS1 - miller dieker - chr 17 - 4 layer classic lissencephaly -
-usually sporadic LIS1 deletion; if familial is AD b/c lose one copy of LIS1 on Chr 17 and get disease
Type 1 - facial dysmorphism, 17p deletion
type 2 - hydrocephalus, dysgenesis cerebellum - Walker Warburg

DCX - x linked lissencephaly - doublecortin
female: subcortical band heterotopia (have all 6 layers)

Periventricular nodular heterotopia- X linked FLNA gene - cytoskeleton stabilization - females X linked dominant

Polymicrogyria: CMV
-GPR56 gene
Zellweger’s

198
Q

grading germinal matrix hemorrhage

A

Premies <32 weeks or low birth weight, germinal matrix bleeding btw caudate + thalamus

I: blood at germinal matrix - good prognosis
II: blood fills ventricles but normal size - good prognosis
III: blood dilates ventricles - poor prognosis
IV: parenchymal hemorrhage from venous infarction - poorest prognosis (dilated ventricles compress veins)

199
Q

Embryonal tumors

A

aggressive, grade IV

  1. Medulloblastoma
  2. Embryonal tumor with multilayered rosettes - ETMR + C19MC
    -pseudostratified epithelium with central clear lumen - heterogenous contrast enhancement, diffusion restriction
    -frontal, parietotemporal
    -ages <5
    -C19MC amplification (non-medulloblastoma)
  3. if no C19M classification but has pseudostratefied neuroepithelium -> medulloepithelioma
  4. atypical teratoid/rhabdoid - INI1 loss - inactivation sMARCB1 on Chr 22 or BRG1 loss

Tx: resection, chemo +/- radiation

200
Q

visual evoked potentials MS - what latency is abnormal? where generated? Optic neuritis on OCT

A

p100 prolonged 120 msec in bad eye
-VEPs sensitive not specific for optic neuritis

from occipital cortex- striate + prestriate

OCT: retinal nerve fiber atrophy associated with cerebral atrophy, disability, decrease visual function (increase fiber layer = edema)

201
Q

wernicke korsakoff

A

Sx: can be VERTIGO or HEARING loss, not always EOM abnormality and gait ataxia
-peripheral nerupathy - LE
-pts with cancer

Damages anterior nuc thalamus in Papez circuit
Hippo ForM ANT CEH
hippocampus to fomix to mamillary body -> mamillilothalamic fibers - > ANT -> cingulate -> entorhinal -> hippocampal formation

Korsokoff - antero+retrograde amnesia - MEdial temp lobes

202
Q

cerebellum structure

A

3 layers: molecular stars in a basket, Purkinje, granular ground of sparks
Superficial layer - Molecular - stellate, basket - inhibit purkinje cells
Middle - Purkinje - inhibitory cells
Deep layer - Granular cell - excitatory

all neurons INHIBITORY except granular cells

203
Q

polymyalgia rheumatica, giant cell / temporal arteritis

A

5-30% get temporal arteritis
-swollen chalky white optic disc
-Most common Sx: HA, shoulder/pelvic girdle pain, fatigue/malaise; jaw claudication in 40%, less likely fever
biopsy: mononuclear infiltrate, intimal thickening + luminal stenosis
doppler ultrasound: halo sign (hypoechoic around artery 2/2 edema)
Tx: steroids; long term Tx Tocilizumab - anti-IL6
-give calcium + vit D b/c chronic steroids

204
Q

wilson

A

ATP7B
Kayser fleisher - limbus of cornea
-urinary copper high

205
Q

familial transthyretin amyloidosis

A

AD, TTR-mediated amyloidosis - pattiesserie -

patisiran, inotersen, vutrisiran - inhibit hepatic synthesis TTR
-Tx peripheral neuropathy

tafamidis - cardiomyopathy

206
Q

Bickerstaff vs autoimmune encephalitis vs Susac

A

bickerstaff - bilateral ophtahmoparesis

susac - autoimmune vasculopathy - thromboses brain, retina, inner ear
-branched retinal A occlusions
-MRI: holes in corpus callosum

207
Q

C6 vs C7 radic

A

C6 - thumb and 1st digit sensory loss, lose biceps and brachioradialis reflex
C7 - middle finger sense loss, triceps weak

208
Q

brain iron accumulation

A

dytonia , movment disorders

vs. hepatolenticular degeneration - Wilson’s

209
Q

personality disorders histrionic vs borderline ; OCPD vs OCD

A

cluster b - dramatic/emotional/erratic- antisocial, borderline, histrionic, narcissistic
histrionic - hypersexual, perceives relationships as more intimate than they are - tearful when denied something
borderline - violent - splitting when denied something
narcissistic - when denied, anger, aggression

OCPD.- miserly, rigid, control issues,

210
Q

cerebral amyloid angiopathy

A

APO E gene mutations

211
Q

KRIT1

A

hereditary cavernomatosis
-recurrent hemorrhages + headaches
(aunt chris)

212
Q

critical illness myopathy histo

A

myosin loss

213
Q

donepezil

A

prolonged QT syndrome

214
Q

toluene vs heroine vs ketamine vs NO, vs Curare

A

toluene: euphoria, hallucinate, depression, nystagmus, ataxia, turn skin grey

inhaled heroine - chasing the dragon - ideomotor apraxia, depression abulia, leukoencephalopathy

-Tx opioid withdrawal-mu opioid agonist - methadone, buprenorphine.- partial mut R agonist-agonist
-precedex - clonidine - alpha 2 agonist (blocks release NE, suppresses autonomic Sx of withdrawal)
(midodrine alpha1 agonist)

NO - laughing gas - NMDA antagonist
-precipitate b12 deficiency

Curare poisoning- mimic locked in - paralysis skeletal muscles

215
Q

brown sequard

A

ipsilateral Horner’s
-ipsilateral vib, proprio loss, strength loss
-contralateral pain temp loss

216
Q

REM parasomnias

A

REm sleep behavior
nightmares
sleep related graoning

217
Q

grading concusion, diffuse axonal injury

A

concussion - no LOC
cerebral contusion - <6 hr LOC

grade 1 diffuse axonal injury - mild - microscopic
Mild DAI: LOC <24 hr, chance good recovery 60%

grade 2 - focal lesions corpus callosum
moderate DAI: LOC >24 hrs, good recovery at 3 months 40%

grade 3 - brainstem microhemorrhages
severe DAI - LOC for days chance good recovery 15% at 3 month
-decorticate posturing

218
Q

paraneoplastic ab

A

parkinsonism-BG-
anti-CRMP5, Ma2, NMDAR,GAD-65

anti-Ta (Ma2) - limbic encephalitis -testicular cancer
-medial temporal lobe hyperintensity
MA will point out the 5th limb (LIMBIC)(elephant penis) next to testicles

SCLC:
- CRMP5 - limbic encephalitis ; cerebellar degen.
-Anti Hu - peripheral neuropathy ; encephalitis
-ACHR-R nicotinic Ab - autonomic Sx, neuropathy- dilated pupils

Isaac syndrome - voltage gated potassium channel - neuromyotonia
-carpopedal spasm, sweating

opsoclonus: anti-Ri - neuroblastoma
You’re anti Rihanna- dancing eyed Jerk!! Blast your neurons!

anti-synthetase syndrome - anti-Jo-1-mechanic’s hands, Raynaud’s
anti-synthetase-anti-PL7/PL12, OJ, EJ

dermatomyositis - Mi2,
TIFI-y, NXP-2-cancer
MDA-5-ILD
-screen CT abdomen/pelvis

“polymyositis”-ILD-CT chest, elevated aldolase

immune mediated necrotizing- super high CK
anti-SRP (myocarditis), anti-HMG-CoA reductase, antimitochondrial

219
Q

aseptic meningitis infectious cause Mollaret VS VZV

A

Mollaret - HSV 2 - 3 episodes of fever + meningismus, lymphocyte predominant
- don’t need to have genital lesions

Dx: HSV 2 PCR in CSF; high lymphocyte WBC, ~normal protein, glucose, no RBCs

vs HSV1-MCC fatal sporadic viral ENCEPHALITIS (hemorrhagic temporal lobe)
-consider NMDA R encephalitis if worsen after HSV-1 encephalitis

vs VZV - vasculitis, SAH, aneurysm

220
Q

glossopharyngeal neuralgia

A

Bradycardia; rare asystole, syncope
-pain at angle of jaw, throat, ear, base of tongue
-triggered by swallowing/yawning
- CN IX into tractus solitarius effect on CN X?

221
Q

alexia without agraphia vs alexia + agraphia

A

alexia without agraphia: corpus callosum lesion, spare angular gyrus

alexia + agraphia: angular gyrus lesion

222
Q

VZV vasculopathy

A

cause: vasculitis large and small arteries, SAH, aneurysm
Dx: VZV IgG Ab in CSF

223
Q

Aromatic acid decarboxylase deficiency

A

AR
-makes DA, epinephrine, NE, 5HT
-Sx: 1st yr life, axial hypotonia, appendicular hypertonia, delay, oculogyric crisis, autonomic crisis*
-Sx worse later in day
-build up Dopa and 3-OmethylDA in blood
-Tx: Da agonist
-usually misdiagnosed

224
Q

dialectal behavioral therapy vs metacognitive therapy

A

metacognitive - look at thought patterns leading to actions
beliefs about your thought content rather than content itself

vs dialectal - treat emotional reactions, relationship difficulty

225
Q

neurohypophysis vs adenohypophysis

A

neurohypophysis: posterior pituitary - neuroectoterm - secretes ADH, oxytocin make from paraventricular/suprachiastmatic hypothalamic neurons

adenohypophysis - anterior pituitary - Rathke pouch

226
Q

most common to least common hypertensive hemorrhage pons, cerebellum

A

putamen (50%), thalamus (15%), pons then cerebellum

227
Q

cerebral palsy hemiplegic vs spastic quadriparesis

A

hemiplegia - perinatal stroke
spastic quadriparesis, dyskinetic CP - perinatal hypoxia

-static disorder - unlikely to progress

228
Q

Dravet treatment stiripentol

A

stiripentol + clobazam - if 6 months
-SE: somnolence, anorexia

stiripentol - GABA R direct allosteric modulator - decrease cell injury after status? neuroprotective?
-inhibits metabolism of clobazam, valproate

229
Q

CMV path vs Rabies path

A

Owl eye - basophilic inclusion body
-cause: retinitis
Tx: ganciclovir

Rabies: negri body (dark spot in periphery of cytoplasm - lighter than inflammatory cells)

230
Q

ADEM

A

monophasic
-young adult after infection
-CSF normal or WBC/protein elevated

231
Q

decort vs decerebrate

A

decorticate: at red nucleus - : disinhibit red nuc, activate rubrospinal tract -> flexor in uppers

decerebrate: between lateral vestibular nucleus (Deiter-caudal pons-maintains upright posture) and red nucleus b/c no inhibition of extension caused by vestibular nucleus

vs injury upper cervical spine/cortex = no posturing

232
Q

SUDEP risk factors

A

highest risk 18-40
kids: if frequent GTCs at night can use nocturnal supervision Or remote listening device

232
Q

more common - hyper or hypokalemic PP?

A

HYPOkalemic PP - most common -CACNA1S usually or SCN4A
-trigger: carbs, exercise

hyperkalemic PP - SCN4 (can get paramyotonia congenital)
-trigger: fasting, exercise

233
Q

herniation syndromes

A

uncal - ipsilateral blown pupil, contralateral hemiparesis OR ipsilateral weakness (Kernohan notch- contralateral peduncle compressed)

subfalcine - leg weakness 2/2 ACA trapped under falx + hydrocephalus

ascending transalar - temporal lobe herniates up across sphenoid ridge; can get MCA / ACA infarct - middle cranial fossa lesion

tonsillar - cushing triad

234
Q

junctional scotoma localization

A

optic N + willebrand knee - contralateral nasal retina fibers -> monocular scotoma + contralateral superior temporal field loss

scotoma of Traquair - monocular temporal hemifield cut from ipsilateral nasal retinal fibers anterior to optic chiasm

235
Q

jugular foramen nerves compression syndromes

A

Vernet: CN 9-11 - intracranial lesion compressing (Vascularis 10, 11 and Nervosa 9)

Collet-Sicard: CN 9-12 - tongue deviate, atrophy - extracranial lesion invading

Tapai: CN 10, 12, 11, Horners - oral intubation, mets, carotid dissection

pars nervosa-9, inferior petrosal sinus
pars vascularis - jugular bulb - 10, 11

pars vascularis - **jugular bulb, internal jugular vein, CN X, IX, ** auricular from X, post. meningeal branch ascending pharyngeal A

236
Q

cortical basal degeneration (astrocytic plaques) vs PSP (tufted astrocytes) vs MSA (glial inclusions)

A

CBD: neuroimaging MOST helpful to Dx - asymmetric atrophy frontoparietal
-4R hyperphosphorylated tau in neurons + glia, astrocytic plaques
-widespread neurodegernation, hyperphosphylated tau
MRI: midcalosal atrophy, asymmetric atrophy parieto-frontal cortex

PSP: tufted astrocytes (astrocytes with tau)

MSA: α-syn within oligodendrocytes- glial cytoplasmic inclusions

237
Q

myotonic dystrophy inheritance 1 vs 2

A

Autosomal dominant
Type 1 - anticipation - CTG repeats in DMPK dystrophia myotonica protein kinase on Chr 19
-posterior subcapsular cataracts
-hypersomnolence, central sleep apnea, baldness, cardiac conduction defect

Type 2 - CCTG repeat on zinc finger 9 on chr 3-autosomal dominant
-no systemic involvement
-no anticipation

238
Q

AIDP with HIV

A

lymphocytic pleocytosis with AIDP

-AIDP not associated with HIV -> albuminologic dissociation

still get absent F waves
-don’t see viral particles on biopsy - see demyelination

239
Q

progressive encephalomyelitis with ridigity and myoclonus

A

PERM - encephalopathy
-autonomic instability
usually autoimmune
-West nile/brucellosis post infectious
-thymoma association

multiple Ab pargets-gad 65, glycine, DPPX, amphiphysin
(stiff person syndrome has no encephalopathy

240
Q

cluster HA duration and treatment

A

15 minutes to 3 hours
-parasympathetic activation causes Sx
Tx: verapamil
add on topiramate

241
Q

congenital myopathies - Nemaline

A

nemaline - early respiratory failure, face neck flexors, proximal muscle weakness early in disease

242
Q

epilepsy genes

A
  1. Benign idiopathic neonatal convulsion
    fifth day fits, seizure 4-6 days
    -variant theta rhythm on EEG
    -clonic seizures
    -KCNQ2
  2. Benign familial neonatal seizures
    KCNQ2, KCNQ3 gene, AUTOSOMAL DOMINANT
    -EEG NORMAL, MRI normal, normal growth/dvelopment

Tx: no tx or oxcarb, carbamazepine

  1. benign familial infantile seizures; SCAN2A, SCAN8A - Autosomal dominant
    -start at 6 months and seizures end at 2 years
    -association with adult paroxysmal kinesigenic dyskinesia in adolescence-PRRT2 gene - proline rich transmembrane protein 2 gene - Chr 16 p

febrile seizures-GEFS+. SCN1A

autosomal dominant nocturnal frontal lobe epilepsy - CHRNA4 gene - alpha 4 subunit nicotinic acetylcholine receptor - seizures out of SLOW WAVE SLEEP - (CHERNING nicotine) hypermotor seizures
-Tx: carbamazepine

JME - GABRA1 - Gaba A receptor
acquired epileptic aphasia Landau Kleffner - NMDA R
Glucose transport type 1 deficiency - SLC2A1 gene - infancy, seizures, cognitive impairment, motor incoordination

SCN1A-Dravet

pyroxine dependent neonates - alpha aminoadipic semialdehyde dehydrogenase - antiquin

243
Q

tx post herpetic neuralgia

A

gabapentin, pregabalin

NOT carbamazepine/LTG

244
Q

MS supportive drugs

A

Dextromethorphan-quinidine - pseudobulbar affect (lack of facial expression)-NOT pseudobulbar palsy
-dextromethorphan = NMDA glutamate R antagonist, 5-HT, NE reuptake inhibitor
-quinidine given to block hepatic metabolism of dextromethorphan

dalfampridine - interacts voltage gated potassium channels
walking in MS

245
Q

reflex bradycardia response sensory

A

general visceral afferents

246
Q

sleep eeg by age

A

Spindle-K-Post
2 months - sleep spindles first appear, asymmetric, asynchronous

2 yr - sleep spindles synchronous

3-6 - K complexes, clear sleep spindles

6 yrs - POSTS, 14+6 positive spikes

247
Q

facioscapulohumeral muscular dystrophy

A

deletion D4Z4 region
-AUTOSOMAL dominant chr 4
-can’t bury eyelashes, scapular winging
-RETINAL Vasculopathy - 50% -
-RESPIRATORY involvement -BL PFTs
-screen hearing loss (common esp if large deletion)
-MSK pain

248
Q

artery adamkiewicz origin
CST lateral vs anterior

A

T8-L2

anterior CST: axial muscles ipsilateral, MOST ventral, more than anterior spinocerebellar tract
vs lateral CST

249
Q

primary enuresis vs secondary

A

for kids 5 yrs and up
primary monosynaptic - never achieved dryness and older than 5 yrs -
-2x more common in boys
Tx: motivational therapy, beahvioral interventions, enuresis alarms first line
high rate spontaneous resolution

nonmonosynaptic - urinary urgency, daytime incontinence, pain, frequency

250
Q

HIV- HAND

A

confluent subcortical T2 hyperintensity - no enhancement
HIV associated neurocognitive disorder

-mild subcortical dementia
-psychomotor slowing

251
Q

dengue fever neuro manifestations

A

rare, 1% of cases
1. transverse myelitis
2. ADEM
3. GBS
mononeuropathies, polyneuropathies
DEN1 Ab
thrombocytopenia
PCR in CSF

252
Q

her

A

in pts with DM, still exclude other causes, can have additional cause in up to half of pts

most common EMG - distal axonopathy

253
Q

thyroid eye disease

A

thyroid ophthalmopathy/orbitopathy
TSH-Ab - Graves
proptosis, chemosis, injection to eyes, tearing, conjection, lid retraction, diplopia b/c big extraocular muscles, -> compressive optic neuropathy

less common - TPO Ab - Hashimoto’s

254
Q

monomelic neuropathy vs monomelic amyotrophy vs neuralgic amyotrophy

A

ischemic monomelic neuropathy - AV shunt placement; weakness not in nerve distribution

monomelic amyotrophy - Hirayama disease - LMN - distal UE weakness - Asian, no sensory Sx

255
Q

pseudodementia memory

A

spared: implicit memory - Ex riding a bike, making a favorite meal

affects semantic memory, episodic memory, remote + working memory

256
Q

stroke + triptans + MOA

A

contraindicated
triptan: 5HT1b 5HT1d agonist

257
Q

ventromedial PFC vs ACC

A

VMPFC: confabulation
disinhibition
emotional lability

ACC - abulia

supplementary motor cortex - mutism, akinesia

amygdala hyperactivity- anxiety - hy

258
Q

IBM Ab

A

NT5C1A protein autoantibody

259
Q

conductive aphasia

A

no repetitive
arcuate fasciculus

260
Q

Waldenstrom macroglobulin vs MM vs MGUS vs IgM paraproteins anti-MAG antibodies

A

waldenstrom - IgM

M spike Multiple Myeloma - IgG

MGUS - IgG or IgA

IgM paraproteins - anti-MAG-distal acquired demyelianting sensory polyneuropathy -acquired stocking gloves and feet neuropathy

261
Q

KCNA1

A

Mutations in KCNA1 have been associated with a variety of human diseases, including:
Myokymia with periodic ataxia (AEMK)
Episodic ataxia type 1 (EA1)
Neuronal developmental disorders
Cardiac dysfunction
Familial paroxysmal kinesigenic dyskinesia
Malignant hyperthermia (MH)
Epilepsy
Hypomagnesemia

262
Q

unresponsive wakefulness vs minimally conscious state vs coma

A

minimally conscious: awareness of self or environment (even if inconsistent)

unresponsive wakefulness (vegetative) - spontaneous eye opening, no purposeful behavior

coma - no eye opening no wakefulness

263
Q

primary, secondary vesicles

A

mesencephalon- midbrain, cerebral peduncle
rhombencephalon = metencephalon (pons, cerebellum, upper 4th ventricle)+ myelencephalon (medulla, lower 4th ventricle)

mesencephalon - midbrain + cerebral peduncle

prosecephalon - telecephalon (hemispheres, BG, lateral ventricles), diencephalon

264
Q

epilepsy with myoclonic-atonic seizures

A

Doose syndrome - Doose is loose
- age 1-5 yrs
-2-5 Hz spike wave

265
Q

CJD

A

total tau elevated in CSF (in AD - low amyloid beta, high phosphorylated tau)

-MRI has DWI cortical ribboning - most patients have DWI abnormality
-DWI abnormality - basal ganglia

typical EEG: 1 Hz periodic BL **sharp wave complexes **discharges/sharp wave complexes
-NOT monophasic

266
Q

Thalamic nuclei + hearing

A

organ of Corti to ipsilateral cochlear nuclei to contralateral inferior colliculus via lateral lemniscus -> BL projections to
medial geniculate nucleus -> superior temporal gyrus aka primary auditory cortex

inferior temporal gyrus - visual processing

superior colliculus - eye movements

267
Q

progressive myoclonic epilepsies

A
  1. Unverricht-Lundborg - EPM1 - (Baltic sea)- cystatin B - chr 21
    -generalized discharges
    -6-15 yrs, stimulus sensitive myoclonus
  2. Lafora -EPM2A deletion - AR - Chr 6
    SKIN biopsy -PAS positive intracellular dots
    -present 12-17 yrs
    -occipital spike wave discharges
  3. MERRF - mitochondrial
  4. neuronal ceroid lipofusinosis - blind, dementia, facial dyskinesias as adult
    CLN1-3, PPT1, TPP1 genes
    -present any age
  5. sialidosis - NEU1 - NEW FARM SILO - cherry red spot - alpha-N acetyl neuraminidase deficiency >mucopolysaccharidosis
    - present 1st yrs of life
    -Dx: urinary sialyl olicosaccaride, lymphocytic vacuolation
268
Q

inhibitory neurotransmitters of CNS, spinal cord

A

glycine - principal inhibitory neurotransmitter of spinal cord
-chloride enters, causes inhibitory postsynaptic potential

GABA - principal inhibitory neurotransmitter everywhere in CNS but spinal cord

269
Q

facial dermatomes

A

v1 - middle of nose

v2 - top lip, lateral nose, - infraorbital, zygomaticofacial, mygomaticotemporal (squidward nose)

v3 - bottom lip -mentalis,
-buccal, auriculotemporal, mental branches

c2-mandible angle, superior occiput like a wrap around the ear - greater auricular nerve

270
Q

leg dermatomes

A

heel - L5 (sliver of medial heel s2)- 1st, 2nd third toes - L5

big toes, medial leg below knee - L4

pinkie toe , lateral foot- S1

buttocks, post, leg - posterior popliteal fossa - S2

271
Q

antiemetics for migraine

A

Tx: metoclopramide (Reglan), chlorpromazine (thorazine/1st gen low potency antipsychotic like thioridazine); prochlorperazine (Compazine for nausea)

No evidence for migraine: ondansetron (Zofran)

272
Q

Midbrain syndromes

A

Weber syndrome- Weak- ventromedial midbrain lesion, ipsilateral cranial nerve (CN) III palsy, contralateral hemiparesis
-motor=medial

Claude syndrome - Clusmy - dorsal midbrain tegmentum lesion, ipsilateral CN III palsy, contralateral cerebellar ataxia, red nucleus

Benedikt syndrome- Both-WEAK + ataxia/tremor; midbrain tegmentum lesion, ipsilateral CN Ill palsy, contralateral hemiparesis, contralateral cerebellar ataxia/ tremor/ choreoathetosis

Dorsal midbrain syndrome, aka Parinaud syndrome: midbrain pretectum lesion, vertical gaze paresis, light-near dissociation, convergence-retraction nystagmus,
Collier lid retraction

273
Q

spinal cord infarct

A

can start with back pain but is abrupt in onset

T6 above - bradycardia, hypotension, autonomic dysreflexia

sulcocommissural artery-branch of ASA at pons level - anterior CST, reticulospinal
arterial vasocorona - connects ASA/PSA

-Artery Adamkiewicz-C6 and below-bladder incontinence + loss reflexes; dorsal columns spared.

274
Q

toxoplasmosis vs cns lymphoma vs abscess vs tumefactive demyelination

A

toxo: hemorrhagic, multiple components

**PCNSL: Notch sign **- deep depression at tumor margin when contrast enhanced
-no DWI signal, no hemorrhage, lots of edema
-specific to PCNSL, not in GMS

GBM-DWI signal
abscess: homogenous DWI signal

tumefactive demyelination - little edema/mass effect

275
Q

muscle fiber types + sensory

A

1a-muscle spindles-myotatic stretch reflex-input to alpha motor neurons-reflex
1b-golgi
II-pacisian, meissier -pressure
III—pain+temp STT three letters
C fibers-small unmyelinated - c -pain

gamma motor neurons-innervate intrafusal fibers-proprioceptive-muscle stretch

276
Q

Tx for LEMS and MOA

A

3,4 diaminopyridine-inhibits presynaptic potassium channels

277
Q

botox type and MOA

A

A, C, E - cleave SNAP 25 (every other)
all the rests - synaptobrevin - B, D, E, F and tetanus toxin

278
Q

organophosphate poisoning

A

impaired ACHestase functioning
-Tx-atropine - binds competitively at muscarinic R (not nicotinic-skeletal muscle)

279
Q

epimysium vs perimysium vs endomysium

A

epimysium - around collection of muscle fascicles (epineurium - multiple fascicles)
perimysium - individual muscle fascicles (perineurium - individual nerve fascicles)
endomysium - muscle fibers

280
Q

neurogenic thoracic outlet

A

medial cord injury
-weaknes to ABP and 1st doral interossei (medial and ulnar muscles mixed) + sensory loss to MEDIAL forearm and hand

vs C6 radic-weakness in the elbow flexors (musculocutaneous biceps)- numbness - lateral hand.

upper trunk lesion would cause weakness in the muscles supplied by the C5/6 roots, including the shoulder and elbow flexors along with numbness in the lateral portion of the arm and hand.

281
Q

L5-S1 paracentral disk hernation

A

L5/S1 level are likely to compress the S1 nerve root, which will exit at the next level below (S1/2 neural foramina). The S1 root innervates the gastrocnemius (elevation onto the toes) and its fibers are responsible for the ankle reflex (hence, ankle reflex would be absent and not preserved). An L5 radiculopathy would cause weakness of great toe extension (EHL), but the L5 nerve root runs laterally to a paracental disc herniation as it exits the L5/S1 neural foramen and thus would be spared.
The L3/4 roots innervate the knee reflex.
The medial calf is saphenous/L4 territory-femoral

282
Q

suprascapular nerve entrapments spinoglenoid

A

suprascapular N at spinoglenoid notch - infraspinatus palsy- just external rotation weakness, spared abduction
suprascapular N at suprascapular notch - supraspinatus - extenal sutation + abduction to 15 weakness

283
Q

conus medullaris vs cauda equina

A

conus - unable to achieve an erection, can’t urinating, decreased sensation around anus but not saddle anesthesia; symmetric heaviness in his legs

cauda equina - saddle anesthesia, PAIN, asymmetric weakness

284
Q

weakness L index finger abduction

A

-distal wound to mid palm
vs:
At Guyon’s canal- ulnar hand muscles + 4/5 digits sensory loss - hypothenar eminence sense spared
-dorsal ulnar cutaneous spared

ulnar neuropathy at elbow: +dorsal ulnar cutaneous + hand muscle weakness

-lower thoracic outlet-C8/T1 medial cord- ulnar and median innervated hand muscles along with sensory loss in the medial hand and forearm.
-scalene triangle fibrous band

-C8 nerve root- weakness of the radial, ulnar and median innervated hand muscles along with sensory loss in the medial hand and forearm.

285
Q

MCC familial ALS + inheritance

A

AD AUTOSOMAL DOMINANT
-C9ORF72-20-40% expansion hexanucleotide
-SOD1 point mutations-10-20% of cases
TDP-43 -familial and sporadic ALS -<5% cases

286
Q

foot drop L5 radiculopathy vs peroneal neuropathy

A

To distinguish:
L5: -hip abduction - L5 nerve root- gluteus medius muscle -superior gluteal nerve
-ankle inversion-posterior tibialis muscle- tibial nerve and supplied by the L5 nerve root (tibial N larger terminal branch sciatic N L4-S3 vs peroneal L4-S2)
[hip adduction obturator L2-L3 adductor magnus]
[peroneal+L5-1st toe extension-extensor hallicis deep fibular nerve, weak in L5 radiculopathy]

L4-tibialis anterior - deep peroneal

only motor branch of common peroneal: short head biceps femoris

L5 nerve root, - gluteus medius; tensor fascia lata (superior gluteal nerve)

To distinguish peroneal vs S1: ankle plantar flexion - S1 tibial
S1 nerve root- gluteus maximus

287
Q

congenital myasthenic syndromes -

A

AR except slow channel - AD

COLQ- slow/absent pupillary response; EOM abnormalities

288
Q

DADS polyneuropathy

A

glove and stocking distribution numbness and unsteady, prolonged distal latencies on NC
+anti-myelin associated glycoprotein-
anti-MAG Ab + IgM paraproteins

289
Q

L3/4 radiculopathy vs femoral N injury

A

Hip adduction - obturator - L3/4 action
extension at knee weak

(hip abduction L5)

290
Q

ganglioglioma vs gangliocytoma staining

A

ganglioglioma: ganglion + glial cells
+GFAP (atrocytes - also GBM) , synaptophysin, CD34 (neuronal)
-temporal lobe

gangliocyoma: no +GFAP staining, only ganglion cells no glial cells

291
Q

motor evoked potentials

A

stimuli-transcranial magnetic stimulation to primary motor cortex

voluntary contraction-facilitation: increase MEP amplitude, shorter onset latency, prolonged potentials

292
Q

pseudobulbar palsy vs progressive bulbar palsy vs pseudobulbar affec

A

psuedobulbar palsy: CN dysfunction outside brainstem, UMN pattern signs

progressive bulbar palsy: degeneration neurons in brainstem motor nuclei

pseudobulbar affect - supratentorial difficulty emotional control

293
Q

MRIs and implantable pacemakers

A

increase serum trop if pacemakesr b/c heating effects-electric currents

-decreases battery life when exposed to magnetic field
-can’t get MRI if DBS, cochlear implant unless MR safe/conditional

294
Q

Perinatal stroke

A

arterial stroke-most common 1st few days of life - focal seizures

venous infarct - periventricular venous infarct - preterm, in utero

295
Q

aica

A

middle cerebellar peduncle, CN VII fascicles, hearing loss

296
Q

receptive aprosody localize

A

difficulty understanding prosody/intonations of speech - right temporoparietal

297
Q

predictive factors of malignant post stroke edema

A

DWI with infarct 82 ml or larger = clinically significant swelling

or NIHSS>15 nondominant and >20 in dominant

298
Q

posterior cortical atrophy

A

Tauopathy with Gerstman syndrome or Balint syndrome, alexia, apraxia, VF defects
-episodic memory spared vs Alzheimer’s
MRI - BL but can be asymmetic - parietal/occipital; temporo-occipital atrophy

299
Q

Intraventricular mass DDx

A

-colloid cyst -MONRO- single layer columnar epithelium, makes mucin, homogeneously enhancing on FLAIR
-Sx: obstructive hydrocephalus
[Monro: btw lateral + 3rd ventricle at level of thalamus]

central neurocytoma - round nuclei uniform cells, small cytoplasm
-lateral ventricles or foramen on Monro
-heterogeneous on imaging vs colloid cyst

choroid plexus papilloma - papillary structure with fibrovascular core lined by cuboidal epithelium
-adults - 4th ventricle
-kids - lateral ventricle

subependymoma - clusters of small nuclei in fibrillary background -scattered microcysts
-4th ventricle, lateral ventricles

subependymal giant cell astrocytoma - TSC - large polygonal eosinophilic cells

(luschka, magendie off 4th ventricle)

300
Q

salivatory nuclei

A

Pons parasympathetic nuclei
-superior salivatory nucleus (submandibular=submaxillary, sublingual - facial-chora typani carries preganglionic parasympathic fibers) + inferior nucleus (Parotid-otic ganglion-glossopharyngeal)

-stimulated by ACh to produce more saliva

posterior salivatiry nuclesu -

301
Q

nucleus ambiguus - motor nucleus

A

(CN IX) and vagus (CN X) efferents- somatic muscles of the pharynx, larynx, and soft palate. (lesion in Wallenberg = hoarse)

-parasympathetic cardiac inhibition - CN X

301
Q

nucleus tractus solitarius - no motor

A

[Scared in solitary confinement]
Rostral = gustatory-Receive taste inputs VII -chorda typani+ IX - (SVA)
Caudal= IX-carotid body+sinus (9 higher than 10)
X-sensory info from organs, vocal folds, aortic arch, aortic body

-baroreceptor reflex- aortic arch and carotid body
Modulate response to hemostatic changes
Participates in limbic system activity
Participates in carotid reflex, gag reflex, cough reflex, and vomiting reflex

chorda typani: taste and preganglionic PNS to submandibular gland

302
Q

hypnic headache treatment AND nummular HA treatment

A

Hypnic: -caffeine 1st line
-2nd line-lithium, indomethacin
NOT melatonin

nummular: gabapentin 1st line (gabapentin as common as coins)

303
Q

pure motor vs pure sensory vs sensorimotor stroke localization

A

pure motor - post limb IC
pure sensory - thalamus
mixed - thalamocapsular

304
Q

neurofibroma vs schwannoma vs ganglioneuromas vs perineuroma

A

neurofibroma: Schwann cells+nerve fibers running through

Schwannomas: only benign Schwann cells, NO nerve fibers running through
perineuroma: perineural cells, pseudo onion bulbing
ganglioneuromas: SYMPATHETIC gangion cells

305
Q

medication overuse HA

A

OTC drugs - 15 days
ergots, triptans, opioids - 10 days

306
Q

CN V (anterior tense) vs CN VII (posterior style)

A

CN V3: Muscles mastication, mylohyoid, TENSE, ANTERIOR digastic
-tensor veli palatini,
-tensor tympani (protects from loud noises)
-mylohyoid
-anterior belly digastric
-touch to anterior 2/3 tongue

CN VII: facial expression, STylohyoid, STapedius, POSTERIOR digastric, chorda typani
-stylohyoid
-stapedius middle ear (protects from loud noises)
-posterior belly digastric
-Chorda typani - taste + preganglionic PNS innervation to superior (gustatory) salivatory nuc
**pterygopalatine=sphenopalatine ganglion - PNS innervation to nasal mucosa, lacrimal gland
-travels through parotid but does not innervate

stylopharyngeus - CN IX

307
Q

CGRP inhibitors

A

erenumab - long term safety and efficacy established - LIBERTY 2021 study -if fail 2-4 preventatives

ubrogepant - CYP3A4 inhibitors contraindicated

308
Q

topiramate

A

levels decrease in pregnancy
-may decrease effectiveness OCPs
-inhibits AMPA, sodium channel blocker, CA blocker

309
Q

Brodmann area primary motor cortex, Broca, Wernicke

A

4: primary motor
9- prefrontal
44, 45: Broca
22: Wernicke

5, 7 - posterior parietal

310
Q

atherosclerosis vs vasculitis vs fibromuscular dysplasia histology

A

atherosclerosis: necrotic core = white, foam cells that are lipids, atheroma cap
-VS arteriolosclerosis: hyaline thickening, NOT lipid deposition

vasculitis - fibrinoid necrosis + sclerosis, NO fatty changes

fibromuscular dysplasia - thickened collagen, muscular hyperplasia no fibrosis

311
Q

should abduction >90 degrees

A

trapezius - spinal accessory nerve C3, C4 - cervical lymph node dissection

serratus anterior - long thoracic -C5-6-7

312
Q

sciatic neuropathy

A

short head+long head biceps femoris weak

short head biceps femoris = common peroneal
long head = tibial division sciatic nerve

Cheng-Ching (17 decks)

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